Interstitial lung disease: Difference between revisions

	Interstitial Lung Disease
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Overview
Classification Fibrosis lung response Occupational lung disease Drug-induced lung injury Radiation-induced lung injury Smoking related interstitial lung disease Idiopathic interstitial pneumonia Pulmonary alveolar proteinosis Lymphocytic infiltrative disorders Pulmonary lymphangioleiomyomatosis Pulmonary hemorrhage syndromes Interstitial lung disease associated with systemic diseases Granulomatous lung response
Pathophysiology
Differentiating Interstitial Lung Disease from other Diseases
Laboratory Finidngs

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Latest revision as of 03:31, 13 February 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2], Anmol Pitliya, M.B.B.S. M.D.[3], Saarah T. Alkhairy, M.D.

Synonyms and keywords: Diffuse parenchymal lung disease; DPLD; ILD

Overview

Interstitial lung disease is a group of disorders involving pulmonary parenchyma. The exact pathogenesis of these disorders is not completely understood. There are multiple initiating factors that may lead to pulmonary injury. However, immunopathogenic responses of lung tissue are quite similar. The major histopathologic patterns in response to lung injury include inflammation, fibrosis and granulomatous response. Interstitial lung disease may be classified into several subtypes based on the lung response to tissue injury and the cause of injury. The underlying cause of interstitial lung disease may include factors such as toxic environmental or occupational exposure, cigarette smoking, and radiation. Interstitial lung disease may also be idiopathic.

Classification

Interstitial lung disease may be classified on the basis of lung response to tissue injury. The lung response to tissue injury may be characterized as:^[1]

Granulomatous
Alveolitis, interstitial inflammation, and fibrosis

Interstitial lung disease

Lung Response:
Granulomatous

Lung Response:
Alveolitis,
Interstitial Inflammation,
and Fibrosis

Known

Idiopathic (Unknown)

Hypersensitivity pneumonitis (organic dusts)

Inorganic dusts

Sarcoidosis

Lymphomatoid granulomatosis

Granulomatous vasculitides

Bronchocentric granulomatosis

Beryllium

Silica

Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome)

Granulomatosis with polyangiitis (Wegener's)

Known cause

Idiopathic (Unknown)

Drug-induced pulmonary toxicity

Occupational and environmental exposure

Radiation-induced lung injury

Aspiration pneumonia

Inhaled inorganic dust

Inhaled organic dusts

Inhaled agents other than inorganic or organic dusts

Desquamative interstitial pneumonia

Respiratory bronchiolitis–associated interstitial lung disease

Pulmonary Langerhans cell granulomatosis

Pulmonary alveolar proteinosis

Idiopathic interstitial pneumonias

Lymphocytic infiltrative disorders
(lymphocytic interstitial pneumonitis
associated with connective tissue disease)

Connective tissue
diseases

Gastrointestinal or
liver diseases

Inherited diseases

Graft-versus-host disease

Pulmonary hemorrhage syndromes

Eosinophilic
pneumonias

Lymphangioleiomyomatosis

Amyloidosis

Major idiopathic interstitial pneumonias

Rare idiopathic interstitial pneumonias

Unclassifiable idiopathic interstitial pneumonias

• Idiopathic pulmonary fibrosis
• Idiopathic nonspecific interstitial pneumonia
• Respiratory bronchiolitis-interstitial lung disease
• Desquamative interstitial pneumonia
• Cryptogenic organising pneumonia
• Acute interstitial pneumonia

• Idiopathic lymphoid interstitial pneumonia
• Idiopathic pleuroparenchymal fibroelastosis

• Systemic lupus erythematosus
• Rheumatoid arthritis
• Ankylosing spondylitis
• Systemic sclerosis
• Sjögren syndrome
• Polymyositis
• Dermatomyositis

• Crohn disease
• Primary biliary cirrhosis
• Chronic active hepatitis
• Ulcerative colitis

• Tuberous sclerosis
• Neurofibromatosis
• Niemann-Pick disease
• Gaucher disease
• Hermansky-Pudlak syndrome

• Bone marrow transplantation
• Solid organ transplantation

• Goodpasture syndrome
• Idiopathic pulmonary hemosiderosis
• Isolated pulmonary capillaritis

Pathophysiology

Interstitial lung disease is a group of disorders affecting pulmonary parenchyma.
The exact pathogenesis of these disorders is not completely understood.
There are multiple initiating factors that may cause pulmonary injury. However, immunopathogenic responses of lung tissue are quite similar.
There are two major histopathologic patterns in response to lung injury which include:
- Inflammation and fibrosis pattern
- Granulomatous pattern

Algorithm showing pathophysiology of Interstitial Lung Disease^[2]

Tissue injury in lungs

Parenchymal injury

Vascular injury

Mast cells in lungs in response to tissue injury

LPA6, LPA2, and LPA4 receptors^[3]

Decreased sFRP-1 (secreted frizzled-related protein 1) in fibroblasts^[4]

Secretes tryptase

Transforming growth factor-β (TGF-β)^[5]

Insulin-like growth factor (IGF) signalling^[4]

Reduced expression of angiogenic factors,
vascular endothelial growth factor (VEGF)^[6]

Elevation of angiostatic factors,
pigment epithelium-derived factor^[7]

Wnt/β-catenin signalling pathway^[8]^[9]

PAR-2/protein kinase (PK)C-α/Raf-1/p44/42 signaling pathway^[10]

Upregulation of Egr-1 (early growth response protein 1)^[11]

IGF-binding protein 5 (IGFBP-5)^[12]

IGF-binding protein 3 (IGFBP-3)

Loss of endothelial barrier function

Dysregulation of repair in lung tissue and activation of fibroblasts^[13]

Regulates transforming growth factor-β (TGF-β)

Induction of syndecan-2 (SDC2)^[14]

Activation,proliferation, and migration of fibroblast to the site of injury

Fibroblasts

Altered PTEN (phosphatase and tensin homologue)/Akt axis

Acquire contractile stress fibres

Inactivates Fox (forkhead box) O3a^[15]

Protomyofibroblast, composed of cytoplasmic actins

Pleural mesothelial cells (PMCs)^[16]^[17]

Downregulation of caveolin-1 (cav-1) and Fas expression^[18]

De novo expression of α-smooth muscle actin (α-SMA)

TGF-β1-dependent mesothelial–mesenchymal transition

Fibroblast resistant to apoptosis^[19]

Myofibroblasts^[20]

Different ranges of contractions mediated by RhoA/Rho-associated kinase

Changes in intracellular calcium concentrations

Recruitement of fibrocytes in lungs

Lock step mechanism of cyclic and contractile events^[21]

T-helper cell type 2 on site of injury^[22]^[23]

Upregulation of C-X-C chemokine receptor type 4 (CXCR4)
on fibrocytes and its ligand
CXCL12 (stromal cell-derived factor 1)^[24]

Excess extracellular matrix production

Exerting traction force

Interleukin-13

Migration of fibrocytes to the site of injury^[25]

Tissue remodelling^[26]

Alternate pathway activation of macrophages^[27]

Lung Fibrosis

Differentiating Interstitial Lung Disease from other Diseases

To review the complete differential diagnosis of dyspnea, click here.

To review the complete differential diagnosis of hemoptysis, click here.

To review the complete differential diagnosis of restrictive lung disease, click here.

Abbreviations: ABG: Arterial blood gas, BAL: Bronchoalveolar lavage, ESR: Erythrocyte sedimentation rate, CRP: C–reactive protein, FVC: Forced vital capacity, RV: Residual volume, FEV1: Forced expiratory volume during the 1st second, DLCO: Diffusing capacity of the lungs for carbon monoxide, O2: Oxygen, TLC: Total lung capacity, PaO2: Arterial partial pressure of oxygen, FiO2: Fraction of inspired oxygen, LDH: Lactate dehydrogenase, CEA: Carcinoembryonic antigen, Anti-GBM antibody: Anti-glomerular basement membrane antibody, A−a gradient: Alveolar-arterial gradient, PAS: Periodic acid-Schiff stain, LAM: Lymphangiomyomatosis, IgE: Immunoglobulin E, ANCA: Anti-neutrophil cytoplasmic antibody, RBC: Red blood cell, ACE: Angiotensin-converting enzyme

Disease		Clinical manifestation															Investigations
		History							Symptoms				Physical examination				Lab findings	Imaging	Pulmonary function test		Bronchoscopy and BAL	Gold standard
		Duration	Age	Gender	Family history	Smoking history	Environmental exposure	HIV	Dyspnea	Cough	Wheezing	Chest pain	Tachypnea	Auscultation	Cyanosis	Clubbing	Lab findings	Imaging	Spirometry	ABG	Bronchoscopy and BAL	Gold standard
Idiopathic pulmonary fibrosis^[28]		Chronic	60−70 years old	Men	+	+	±	−	+	Dry	+	+	+	Inspiratory high−pitched rhonchi Bibasilar inspiratory crackles	−	+	Antinuclear antibody + Rheumatoid factor + Elevated ESR Elevated CRP Polycythemia	Bibasilar, peripheral reticular abnormalities Focal honeycomb cyst formation Traction bronchiectasis	↓ FVC ↑ RV Normal FEV1/FVC ↓ DLCO	↓ O2	Not required ↑ Neutrophils ↑ Eosinophils	Diagnosis of exclusion Lung biopsy
Idiopathic nonspecific interstitial pneumonia^[29]		Acute/Chronic	50−60 years old	Female	+	−	−	+	+	+	+	−	+	Bibasilar crackles	−	±	Normal	Bilateral ground−glass opacities Fine reticular infiltrates Traction bronchiectasis Consolidation	Normal to ↑ FEV1/FVC ↓ FVC ↓ TLC	↓ O2	Nonspecific	Lung biopsy and multidisciplinary approach
Cryptogenic organizing pneumonia^[30]		Acute/subacute	50−60 years old	Both	−	±	−	−	+	Dry	−	−	−	Inspiratory crackles	−	−	Leukocytosis Elevated ESR Elevated CRP	Alveolar filling and air bronchograms Bilateral ground−glass opacities Bilateral consolidation	↓ FVC Normal FEV1/FVC ↓ DLCO	↓ O2	↑ Lymphocytes ↑ Neutrophils ↑ Eosinophils	Lung biopsy
Disease		History							Symptoms				Physical examination				Lab findings	Imaging	Pulmonary function test		Bronchoscopy and BAL	Gold standard
Disease		Duration	Age	Gender	Family history	Smoking history	Environmental exposure	HIV	Dyspnea	Cough	Wheezing	Chest pain	Tachypnea	Auscultation	Cyanosis	Clubbing	Lab findings	Imaging	Spirometry	ABG	Bronchoscopy and BAL	Gold standard
Acute interstitial pneumonia (Hamman−Rich syndrome)^[31]		Acute	50−60 years old	Both	−	−	−	−	+	+	−	−	+	Diffuse crackles	−	−	Leukocytosis	Bilateral and symmetric, diffuse ground glass Alveolar consolidation opacities Traction bronchiectasis Honeycomb fibrosis	N/A	↓ O2 PaO2/FiO2 <200 mmHg	Nonspecific ↑ Neutrophils ↑ Atypical epithelial cells	Lung biopsy
Lymphocytic interstitial pneumonia^[32]		Subacute	30−40 years old	Female	−	−	−	±	+	+	+	+	−	Diffuse crackles	−	+	Gammopathy	Diffuse ground glass attenuation with fibrosis Centrilobular and subpleural nodules Lung cysts	↓ FVC ↓ TLC ↓ DLCO	↓ O2	Nonspecific ↑ Total BAL cell count BAL lymphocytosis	Lung biopsy
Respiratory bronchiolitis−interstitial lung disease^[33]		Subacute	30−40 years old	Both	−	+	−	−	+	Dry	+	−	−	Inspiratory high−pitched rhonchi Fine, bibasilar end−inspiratory crackles	−	−	Nonspecific	Diffuse or patchy ground glass opacities in a mosaic pattern Fine nodules Air trapping	↓ FVC ↓ TLC ↓ DLCO	↓ O2	↑ Macrophages	Clinical evaluation and investigations
Desquamative interstitial pneumonia^[34]^[35]		Chronic	40−50 years old	Both	−	+	−	−	+	Dry	+	−	−	Fine, bibasilar end−inspiratory crackles	−	−	Nonspecific	Ground glass opacities without the peripheral reticular and reticulonodular opacities	↓ DLCO	↓ O2	↑ Eosinophils	Lung biopsy
Disease		History							Symptoms				Physical examination				Lab findings	Imaging	Pulmonary function test		Bronchoscopy and BAL	Gold standard
Disease		Duration	Age	Gender	Family history	Smoking history	Environmental exposure	HIV	Dyspnea	Cough	Wheezing	Chest pain	Tachypnea	Auscultation	Cyanosis	Clubbing	Lab findings	Imaging	Spirometry	ABG	Bronchoscopy and BAL	Gold standard
Pulmonary Langerhans cell granulomatosis^[36]		Chronic	20−40 years old	Both	+	+	−	−	±	Dry	+	+	−	Unremarkable	−	−	Nonspecific	Mid to upper lung zone cysts and nodules Reticular and nodular opacities Recurrent spontaneous pneumothorax	↓ FEV1/FVC	Normal	>5 percent langerhans cells (CD−1a positive)	Lung biopsy
Pulmonary alveolar proteinosis^[37]^[38]		Acute/chronic	40−50 years old	Male	+	+	+	−	+	+	+	−	−	Inspiratory crackles	+	+	↑ LDH ↑ CEA ↑ Surfactant protein A, B, and D Anti-GBM antibody + Polycythemia Hypergammaglobulinemia	Bbilateral perihilar and basilar alveolar opacities without air−bronchograms "Bat wing" distribution Intralobular thickening Diffuse ground−glass opacities	↑ FEV1/FVC ↑ A−a gradient ↓ DLCO	↓ PaO2 ↓ O2 Respiratory alkalosis	Large foamy macrophages with amorphous PAS−positive material ↑ Lymphocytes	Bronchoscopy and BAL
Pulmonary lymphangioleiomyomatosis^[39]		Acute/chronic	30−40 years old	Female	+	+	−	−	+	Bloody	+	+	−	Decreased breath sounds	−	+	↑ Vascular endothelial growth factor−D (VEGF−D)	Pneumothorax Chylothorax Thin−walled round cystic lesions	↓ FEV1/FVC	↓ PaO2 ↓ O2	LAM cells +	Lung biopsy
Eosinophilic pneumonia^[40]		Acute/chronic	20−40 years old	Male	−	−	−	−	+	Dry	+	+	+	Decreased breath sounds	−	−	Neutrophilic leukocytosis ↑ Eosinophils Elevated ESR Elevated CRP Elevated IgE level	Bilateral diffuse mixed ground glass and reticular opacities Small bilateral pleural effusions Centrilobular nodules and air−space consolidation	↑ FEV1/FVC ↓ DLCO	↓ PaO2 ↓ O2	Eosinophilia	Clinical evaluation and investigations
Hypersensitivity pneumonitis^[41]		Acute/subacute/chronic	40−60 years old	Both	−	±	+	−	+	Dry/productive	+	+	+	Diffuse fine bibasilar crackles	−	+	Neutrophilic leukocytosis	Centrilobular ground−glass or nodular opacities of mid−to−upper zone Air−trapping	↓ FEV1 ↓ FVC	↓ PaO2 ↓ O2	Lymphocytosis	Lung biopsy
Disease		History							Symptoms				Physical examination				Lab findings	Imaging	Pulmonary function test		Bronchoscopy and BAL	Gold standard
Disease		Duration	Age	Gender	Family history	Smoking history	Environmental exposure	HIV	Dyspnea	Cough	Wheezing	Chest pain	Tachypnea	Auscultation	Cyanosis	Clubbing	Lab findings	Imaging	Spirometry	ABG	Bronchoscopy and BAL	Gold standard
Occupational lung disease^[42]		Chronic	Elderly	Male	+	+	+	−	±	+	+	+	+	Fine crackles	Peripheral/central	+	Anemia Neutrophilia Elevated ESR Elevated CRP Elevated immunoglobulin	Pleural thickening and plaques Calcification Nodular or reticular opacities Lobar consolidation Atelectasis Parenchymal bands Enlarged hilar or mediastinal lymph nodes Granulomata	↑ FEV1/FVC	↓ O2 ↑ CO2 Respiratory acidosis	Mineral dust +	History of environmental exposure and imaging Lung biopsy not required
Radiation−induced lung injury^[43]		Subacute/chronic	Any age	Both	−	−	+	−	+	Dry	+	+	+	Crackles Pleural rub Dullness to percussion	+	−	Nonspecific	Perivascular haziness to patchy alveolar filling densities Straight line effect Pleural effusion	↓ TLC ↓ FVC ↓ FEV1 ↓ DLCO	↓ PaO2 ↓ O2	Lymphocytosis	History of irradiation and clinical presentation
Pulmonary hemorrhage syndromes	Goodpasture syndrome^[44]	Chronic	All ages	Male	+	±	−	−	±	Bloody	±	−	−	Bilateral coarse crepitations	−	−	Anti-GBM antibody + ANCA + Anemia RBC in the urine	Pulmonary infiltrates	↑ DLCO	Normal	NA	Kidney biopsy
	Idiopathic pulmonary hemosiderosis^[45]	Acute/subacute/chronic	Children − 10 years old	Both	+	±	−	−	+	Bloody	+	+	−	Crackles	−	−	Iron deficiency anemia ↑ Plasma bilirubin ↑ Urinary excretion of urobilinogen ↑ Reticulocytes Fecal occult blood +	Mid to lower zone alveolar opacities Multiple honeycomb cysts	↓ TLC ↓ FVC ↓ FEV1 ↑ FEV1/FVC ↑ DLCO	↓ O2 ↓ CO2	↑ Hemosiderin−laden macrophages	Clinical evaluation and investigations
	Isolated pulmonary capillaritis^[46]	Chronic	40−60 years old	Both	+	−	±	−	+	Bloody	+	+	+	Decreased breath sounds	−	−	Anemia Leukocytosis	Diffuse alveolar haemorrhage	↓ FEV1/FVC	↓ O2	Diffuse alveolar haemorrhage	Diagnosis of exclusion
Disease		History							Symptoms				Physical examination				Lab findings	Imaging	Pulmonary function test		Bronchoscopy and BAL	Gold standard
Disease		Duration	Age	Gender	Family history	Smoking history	Environmental exposure	HIV	Dyspnea	Cough	Wheezing	Chest pain	Tachypnea	Auscultation	Cyanosis	Clubbing	Lab findings	Imaging	Spirometry	ABG	Bronchoscopy and BAL	Gold standard
Sarcoidosis^[47]		Acute/subacute/chronic	20−40 years old	Female	+	±	−	−	±	+	+	±	−	Crackles Wheezing Decreased breath sounds	+	−	Hypercalciuria Hypercalcemia High ACE Hypergammaglobulinemia	Hilar adenopathy Reticular opacities Pneumothorax Pleural thickening Chylothorax Pulmonary hypertension	↓ TLC ↓ FVC ↓ FEV1 ↑ FEV1/FVC ↓ DLCO	↓ O2 ↓ CO2 Respiratory acidosis	Lymphocytosis Elevated adenosine deaminase D-dimer +	Clinical evaluation and investigations
Granulomatous vasculitides	Granulomatosis with polyangiitis (Wegener)^[48]	Chronic	Elderly	Both	+	−	−	−	+	+	+	±	−	Crackles	−	−	ANCA + Anemia Leukocytosis Thrombocytosis Elevated ESR Elevated CRP Elevated creatinine Urine protein + Hematuria	Cavitate nodules Ground−glass opacity Consolidation Pleural effusion Hilar adenopathy	↓ FEV1/FVC	↓ O2	Alveolar hemorrhage	Lung biopsy
Granulomatous vasculitides	Eosinophilic granulomatosis with polyangiitis (Churg Strauss)^[49]	Chronic	40−50 years old	Both	+	−	−	−	−	+	+	−	−	Scattered wheezing	−	−	Eosinophilia Elevated IgE titers ANCA +	Areas of parenchymal opacification Mixed interstitial patchy alveolar opacities	↓ TLC ↑ RV	Normal	Eosinophilia	Lung biopsy
Bronchocentric granulomatosis^[50]		Chronic	30−70 years old	Both	−	−	−	−	±	±	+	±	−	Scattered wheezing	−	−	Eosinophilia Elevated IgE titers Elevated circulating IgE antibodies to Aspergillus species	Upper zone single or multiple pulmonary nodules Consolidation Atelectasis	↓ FEV1/FVC	Normal	Eosinophilia	Lung biopsy
Pulmonary lymphomatoid granulomatosis^[51]		Chronic	30−50 years old	Male	−	−	−	−	+	+	+	+	−	Normal	−	−	Epstein-Barr virus (EBV) serology +	Mid to lower zone multiple poorly defined nodules Diffuse reticular abnormalities	Normal	↓ O2 Chronic respiratory alkalosis	Normal	Lung biopsy
Amyloidosis^[52]^[53]		Subacute/chronic	50−70 years old	Male	+	−	−	−	−	Bloody	+	−	−	Crackles	−	−	Congophilia with apple−green birefringence under polarized light	Tracheobronchial infiltration Persistent pleural effusions Parenchymal nodules	↓ FEV1/FVC	↓ O2	Normal	Lung biopsy
Disease		History							Symptoms				Physical examination				Lab findings	Imaging	Pulmonary function test		Bronchoscopy and BAL	Gold standard
Disease		Duration	Age	Gender	Family history	Smoking history	Environmental exposure	HIV	Dyspnea	Cough	Wheezing	Chest pain	Tachypnea	Auscultation	Cyanosis	Clubbing	Lab findings	Imaging	Spirometry	ABG	Bronchoscopy and BAL	Gold standard

Laboratory Finidngs

There are multiple laboratory tests that may be helpful to ascertain or rule out the diagnosis of interstitial lung disease.

Condition	Disease	Investigation
All patients with suspicious interstitial lung disease		Complete blood count and differential
		Liver function tests	Alanine aminotransferase (ALT, SGPT)
			Aspartate aminotransferase (AST, SGOT)
			Alkaline phosphatase
		Renal function tests	Urinalysis
			Blood urea nitrogen (BUN)
			Creatinine (Cr)
Suspicious of systemic rheumatic disease	RA	Serology	Anti−cyclic citrullinated peptide (Anti-CCP)
	SLE	Serology	Anti−double stranded DNA antibodies (Anti-dsDNA antibody)
	Amyopathic dermatomyositis	Serology	Anti-melanoma differentiation-associated gene 5 (MDA-5)
	Nonspecific	Serology	Antinuclear antibody (ANA)
		Serology	Rheumatoid factor (RF)
		Serology	Anti-neutrophil cytoplasmic antibody (ANCA)
		Enzyme	Creatine kinase (CK), aldolase
Mechanic hands	Myositis	Myositis−associated antibodies	Anti-tRNA synthetases Jo-1
			Anti-tRNA synthetases PL-7
			Anti-tRNA synthetases PL-12
Sicca features or positive anti−extractable nuclear antigen (ENA)	Sjögren’s syndrome	Serology	Anti-RO (SS−A)
	Sjögren’s syndrome	Serology	Anti-La (SS−B)
	Mixed connective tissue disease	Serology	Anti-ribonucleoprotein (RNP)
	IgG4-related disease	Serology	Serum IgG4
Severe GERD or sclerodactyly	Limited systemic scleroderma	Serology	Anti-centromere
Severe GERD or sclerodactyly	Systemic scleroderma	Serology	Anti-topoisomerase I (anti-Scl-70)
Dyspnea	Heart failure	Enzyme	Brain natriuretic peptide (BNP)
Dyspnea	Pulmonary hypertension	Enzyme	N-terminal proBNP (NT-proBNP)
Anemia and/or hemoptysis	Coagulopathies	Coagulation studies
	Goodpasture syndrome	Serology	Anti−glomerular basement membrane (GBM) antibodies
	Antiphospholipid syndrome	Serology	Antiphospholipid antibodies
	Idiopathic pulmonary hemosiderosis	Serology	Serum IgA endomysial or tissue transglutaminase antibodies
Mediastinal lymphadenopathy	Multiple myeloma	Serum protein electrophoresis
Beryllium exposure	Berylliosis	Peripheral blood beryllium lymphocyte proliferation test
Risk factors for HIV	HIV	ELISA
Risk factors for HIV	HIV	Western blot test

Occupational Lung Disease

Occupational lung diseases are caused by the accumulation of different dust particles in the alveolar space.^[54]
As the particles accumulate, the elimination mechanisms of the body begin to fail, resulting in activation of chemotactic factors that exacerbate the inflammatory response, and subsequently lead to fibrosis.
The most common particles that cause pneumoconiosis are asbestos, silica, coal, magnesium silicate, aluminum silicate, bauxite, cobalt, beryllium and iron.

For more information about occupational lung disease, click here.

Drug-induced lung injury

More than 600 medications have pulmonary toxicity and may cause lung injury.^[55]

Lung injury following medication intake might vary from interstitial lung disease to hypersensitivity pneumonitis, pleural effusion or pulmonary edema.^[56]^[57]
The presentation of lung injury might be acute, subacute or chronic and it might occur weeks to years even after discontinuing the drug.^[58]^[59]
Diagnosis of drug-induced lung injury is by the exclusion of other diseases. Detailed history and paraclinical pulmonary investigations are required to exclude other causes of interstitial lung disease. Pulmonary investigations are as follow:^[60]
- Restrictive pattern on pulmonary function tests (PFTs)
- FEV1/FVC ratio normal or increased
- Reduced DLCO
- Bronchoalveolar lavage may exclude infectious disease
- Bronchoscopy with transbronchial biopsy may be indicated to exclude other pulmonary diseases
- Invasive lung biopsy rarely required
Management of drug-induced lung injury includes:^[61]
- Immediate drug discontinuation
- Supportive therapy
- Control of other underlying chronic pulmonary disease
- Treatment of respiratory infections
- Smoking cessation
- Glucocorticoid therapy^[62]
List of medications that might cause interstitial lung disease is as follow:^[63]^[64]

Drug−induced lung injury
Antimicrobial Agents	Anti−Inflammatory Agents	Biological Agents	Cardiovascular Agents	Immunomodulator agents	Antineoplastic agents	Miscellaneous
Amphotericin B Doripenem Ethambutol Isoniazid Minocycline Nitrofurantoin Sulfasalazine	Abatacept Aspirin Azathioprine Cyclophosphamide Gold Anakinra Leflunomide Methotrexate Nonsteroidal anti-inflammatory drugs Penicillamine Sulfasalazine Thalidomide	Adalimumab Alemtuzumab Alpha interferon Bevacizumab Cetuximab Etanercept Infliximab Panitumumab Rituximab Tocilizumab Trastuzumab	ACE inhibitors Amiodarone^[65] Anticoagulants Beta blockers Flecainide Hydrochlorothiazide Procainamide Statins^[66] Tocainide	Sirolimus Riluzole Trametinib vandetanib	Azathioprine Bleomycin Bortezomib Busulfan Carmustine (BCNU)^[67] Chlorambucil Colony-stimulating factors Crizotinib Cyclophosphamide^[68] Cytarabine Deferoxamine Docetaxel Doxorubicin Eribulin Erlotinib Etoposide (VP−16) Fludarabine Flutamide Gefitinib Gemcitabine^[69] Hydroxyurea Imatinib Lomustine (CCNU) Melphalan Mitomycin-C Nilutamide Nitrosourea Olsalazine Paclitaxel Procarbazine Semustine (Methyl−CCNU) Sorafenib Vinblastine	Bacille Calmette-Guerin (BCG) Bromocriptine Carbamazepine Cabergoline Cocaine^[70] Methysergide L-tryptophan Penicillamine Phenytoin Talc

Radiation-induced Lung Injury

Radiation has been considered as one of the causes of lung injury. About 5 to 15% of patients receiving radiation therapy may present with pulmonary symptoms.^[71]

Pulmonary injury following irradiation is directly related to duration and dose of radiation.^[72]
The main pathogenesis of radiation-induced lung injury is the damage to the type I pneumocytes which triggers the initiation of reactions. It leads to secretion of growth factors and proteases which increases degradation of extracellular matrix. Also, radiation causes damage to epithelial cells which leads to loss of barrier function. All of these changes cause a cycle of inflammation and fibrosis.^[43]
There are two types of reaction in pulmonary tissues to radiation which include:^[73]
- Early pulmonary reaction:
  - It usually occurs in 4 to 12 weeks following irradiation.
  - Early reaction mostly presents as radiation pneumonitis.
  - Treatment of radiation pneumonitis include steroids, ACE inhibitors and pentoxifylline.
- Late pulmonary reaction:
  - It usually occurs 6 to 12 months following irradiation.
  - Late response usually induces lung fibrosis.
  - Management of lung fibrosis consists of supportive therapy, airway secretions mobilization, anti-inflammatory therapy, and management of acute exacerbations.
CT might be used for the diagnosis of radiation pneumonitis. Ground glass opacities, consolidation, fibrosis, atelectasis, pulmonary volume loss, or pleural thickening might be seen on CT scan.

Smoking related Interstitial Lung Disease

Cigarette smoking may cause various adverse effects on pulmonary tissue.^[74]^[75]^[76]

Cigarette smoke might injure alveolar epithelial cells leading to in diffuse infiltration and subsequently parenchymal fibrosis.
Alveolar epithelial cells are the primary cells involved in the pathogenesis of emphysema as well as lung fibrosis following smoking.
Cigarette smoking may predispose alveolar epithelial cells to cellular senescence and apoptosis that leads to emphysema or lung fibrosis.
A well-known association exists between tobacco smoking and some types of interstitial lung disease such as:
- Desquamative interstitial pneumonia
- Respiratory bronchiolitis-associated interstitial lung disease
- Pulmonary Langerhans cell granulomatosis
- Acute eosinophilic pneumonia

Idiopathic Interstitial Pneumonia

The idiopathic interstitial pneumonias (IIP) are a broad range of interstitial lung diseases of unknown etiology.^[77]^[78]^[79]^[80]^[81]^[82]

Pathogenesis of idiopathic interstitial pneumonia is fibroblasts proliferation and collagen deposition leading to inflammation and fibrosis.
Patients with idiopathic interstitial pneumonia generally manifest as a gradual dyspnea and dry cough.
On chest imaging it is characterized by bilateral abnormal opacities of various types.
The most common type of idiopathic interstitial pneumonia is chronic idiopathic pulmonary fibrosis. However, acute interstitial pneumonia has the worst prognosis.
The exact diagnosis can only be reached with a multidisciplinary approach after excluding known causes.
The new classification of idiopathic interstitial pneumonias is as follow:
- Major idiopathic interstitial pneumonias
  - Idiopathic pulmonary fibrosis
  - Idiopathic nonspecific interstitial pneumonia
  - Respiratory bronchiolitis-interstitial lung disease
  - Desquamative interstitial pneumonia
  - Cryptogenic organizing pneumonia
  - Acute interstitial pneumonia
- Rare idiopathic interstitial pneumonias
  - Idiopathic lymphoid interstitial pneumonia
  - Idiopathic pleuroparenchymal fibroelastosis
- Unclassifiable idiopathic interstitial pneumonias

For more information about Idiopathic interstitial pneumonia, click here.

For more information about idiopathic pulmonary fibrosis, click here.

For more information about Cryptogenic organizing pneumonia, click here.

Pulmonary Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare lung disease that is characterized by the intra-alveolar accumulation of surfactant phospholipid and apoproteins.^[83]^[84]

The exact etiology of pulmonary alveolar proteinosis is unknown.
The primary pathogenesis of pulmonary alveolar proteinosis involves reduction in granulocyte-macrophage colony-stimulating factor (GM-CSF) levels or function and/or impaired alveolar macrophage function.
Terminal bronchioles and alveoli are filled with a lipoproteinaceous material that will be periodic acid-Schiff (PAS) stain positive.
If left untreated, patients with pulmonary alveolar proteinosis may progress to develop pulmonary fibrosis or cor pulmonale.

For more information about pulmonary alveolar proteinosis, click here.

Lymphocytic Infiltrative Disorders

Lymphocytic infiltrative disorders might cause interstitial lung disease in mostly HIV positive children.^[85]^[86]

The etiology of lymphocytic infiltrative disorders is unknown. However, there is an evidence of infectious cause such as EBV in HIV positive patients.
The two main manifestations of lymphocytic infiltrative disorders include:
- Lymphocytic interstitial pneumonitis
- Pulmonary lymphomatoid granulomatosis

For more information about lymphocytic interstitial pneumonitis, click here.

Pulmonary Lymphangioleiomyomatosis

Lymphangiomyocytosis (LAM) is defined as a multifocal neoplasm with differentiation of the perivascular epithelioid cell that involves multiple organs.^[87]

Lymphangiomyomatosis is the result of disorderly smooth muscle proliferation throughout the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax) and lymphatics (leading to chylous pleural effusion).
LAM occurs in a sporadic form, which only affects females, who are usually of childbearing age.

For more information about pulmonary lymphangioleiomyomatosis, click here.

Pulmonary Hemorrhage Syndromes

Pulmonary hemorrhage syndromes might cause interstitial lung disease.

Several pulmonary hemorrhage syndromes that affect the lung parenchyma and eventually lead to pulmonary fibrosis. Some of these are as follows:
- Goodpasture syndrome
- Idiopathic pulmonary hemosiderosis
- Isolated pulmonary capillaritis

Interstitial Lung Disease Associated with Systemic Diseases

Interstitial lung disease associated with connective tissue diseases

Systemic lupus erythematosus
Rheumatoid arthritis
Ankylosing spondylitis
Systemic sclerosis
Sjögren syndrome
Polymyositis/dermatomyositis
Granulomatosis with polyangiitis (Wegener's)
Eosinophilic granulomatosis with polyangiitis (Churg Strauss)

Interstitial lung disease associated with inherited diseases

Interstitial lung disease associated with gastrointestinal or liver diseases

Interstitial lung disease associated with graft−versus−host disease

Granulomatous Lung Response

Hypersensitivity pneumonitis
Sarcoidosis
Granulomatous vasculitides
- Granulomatosis with polyangiitis (Wegener)
- Eosinophilic granulomatosis with polyangiitis (Churg Strauss)
Bronchocentric granulomatosis

For more information about hypersensitivity pneumonitis, click here.

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[pmid25864717-83] Papiris SA, Tsirigotis P, Kolilekas L, Papadaki G, Papaioannou AI, Triantafillidou C, Papaporfyriou A, Karakatsani A, Kagouridis K, Griese M, Manali ED (June 2015). "Pulmonary alveolar proteinosis: time to shift?". Expert Rev Respir Med. 9 (3): 337–49. doi:10.1586/17476348.2015.1035259. PMID 25864717.

[pmid27514590-84] Suzuki T, Trapnell BC (September 2016). "Pulmonary Alveolar Proteinosis Syndrome". Clin. Chest Med. 37 (3): 431–40. doi:10.1016/j.ccm.2016.04.006. PMID 27514590.

[pmid27514593-85] Panchabhai TS, Farver C, Highland KB (September 2016). "Lymphocytic Interstitial Pneumonia". Clin. Chest Med. 37 (3): 463–74. doi:10.1016/j.ccm.2016.04.009. PMID 27514593.

[pmid9363179-86] Fishback N, Koss M (September 1996). "Update on lymphoid interstitial pneumonitis". Curr Opin Pulm Med. 2 (5): 429–33. PMID 9363179.

[pmid29487252-87] Verma AK, Joshi A, Mishra AR, Kant S, Singh A (2018). "Pulmonary lymphangioleiomyomatosis presenting as spontaneous pneumothorax treated with sirolimus - A case report". Lung India. 35 (2): 154–156. doi:10.4103/lungindia.lungindia_60_17. PMID 29487252.

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@@ Line 4: / Line 4: @@
 '''For the WikiPatient page for this topic, click [[Interstitial lung disease (patient information)|here]]'''
-{{CMG}}; {{AE}} Saarah T. Alkhairy, M.D.
+{{CMG}}; {{AE}} {{SSH}}, {{Anmol}}, Saarah T. Alkhairy, M.D.
 {{SK}} Diffuse parenchymal lung disease; DPLD; ILD
 ==Overview==
+Interstitial lung disease is a group of disorders involving pulmonary [[parenchyma]]. The exact pathogenesis of these disorders is not completely understood. There are multiple initiating factors that may lead to [[pulmonary]] injury. However, immunopathogenic responses of [[lung]] tissue are quite similar. The major histopathologic patterns in response to [[lung]] injury include [[inflammation]], [[fibrosis]] and [[Granuloma|granulomatous]] response. Interstitial lung disease may be classified into several subtypes based on the lung response to [[tissue]] injury and the cause of injury. The underlying cause of interstitial lung disease may include factors such as toxic [[Environmental Health Perspectives|environmental]] or [[Occupational Health|occupational]] exposure, [[Smoking|cigarette smoking]], and [[Radiation therapy|radiation]]. Interstitial lung disease may also be idiopathic.
 ==Classification==
-Interstitial lung disease may be classified on the basis of lung response to tissue injury and include:<ref>{{cite book | last = Kasper | first = Dennis | title = Harrison's principles of internal medicine | chapter =315: Interstitial Lung Diseases | editor1-last=Talmadge |editor1-first= E. King, Jr. | publisher = McGraw Hill Education | location = New York | year = 2015 | isbn = 0071802150 }}</ref>
+Interstitial lung disease may be classified on the basis of lung response to [[tissue]] injury. The lung response to tissue injury may be characterized as:<ref>{{cite book | last = Kasper | first = Dennis | title = Harrison's principles of internal medicine | chapter =315: Interstitial Lung Diseases | editor1-last=Talmadge |editor1-first= E. King, Jr. | publisher = McGraw Hill Education | location = New York | year = 2015 | isbn = 0071802150 }}</ref>
-*Lung response: Granulomatous
+*[[Granulomatous]]
-*Lung response: Alveolitis, interstitial inflammation, and fibrosis
+*[[Alveolitis]], interstitial [[inflammation]], and [[fibrosis]]
+<div style="width: 80%">
 <small>
 {{Family tree/start}}
 {{Family tree| | | | | | | | | | | | | | | A01 | | | | | | | | | | | | A01=Interstitial lung disease}}
 {{Family tree| | | | | | | | | | |,|-|-|-|-|^|-|-|-|-|-|-|-|-|.| |}}
-{{Family tree| | | | | | | | | | B01 | | | | | | | | | | | | B02 | | | | | |B01=Lung Response:<br>Granulomatous|B02=Lung Response:<br>Alveolitis,<br>Interstitial Inflammation,<br>and Fibrosis }}
+{{Family tree| | | | | | | | | | B01 | | | | | | | | | | | | B02 | | | | | |B01=Lung Response:<br>[[Granulomatous]]|B02=Lung Response:<br>[[Alveolitis]],<br>Interstitial [[Inflammation]],<br>and [[Fibrosis]] }}
 {{Family tree| | | | | | |,|-|-|-|^|-|-|-|.| | | | | | | | | |!| | | | | | | | | | |}}
 {{Family tree| | | | | | C01 | | | | | | C02 | | | | | | | | |!| | | | | | | | | | | |C01=Known|C02=Idiopathic (Unknown)}}
 {{Family tree| | |,|-|-|-|(| | | |,|-|-|-|+|-|-|-|v|-|-|-|.| |!| | | | | | | | | | |}}
-{{Family tree| | D01 | | D02 | | D03 | | D04 | | D05 | | D06 |!| | | | | | | | | | |D02=Inorganic dusts|D01=Hypersensitivity pneumonitis (organic dusts)|D03=Sarcoidosis||D04=Lymphomatoid granulomatosis|D05=Granulomatous vasculitides|D06=Bronchocentric granulomatosis}}
+{{Family tree| | D01 | | D02 | | D03 | | D04 | | D05 | | D06 |!| | | | | | | | | | |D02=Inorganic dusts|D01=[[Hypersensitivity pneumonitis]] (organic dusts)|D03=[[Sarcoidosis]]||D04=[[Lymphomatoid granulomatosis]]|D05=Granulomatous vasculitides|D06=[[Bronchocentric granulomatosis]]}}
 {{Family tree| | | | |,|-|^|-|.| | | | | | | |,|-|^|-|.| | | |!| | | | | | | | | | |}}
-{{Family tree| | | | E01 | | E02 | | | | | | D07 | | D08 | | |!| | | | | | | | | | |E01=Beryllium|E02=Silica|D07=Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome)|D08=Granulomatosis with polyangiitis (Wegener)}}
+{{Family tree| | | | E01 | | E02 | | | | | | D07 | | D08 | | |!| | | | | | | | | | |E01=[[Occupational lung disease|Beryllium]]|E02=[[Occupational lung disease|Silica]]|D07=Eosinophilic granulomatosis with polyangiitis ([[Churg - Strauss Syndrome|Churg Strauss syndrome]])|D08=[[Granulomatosis with polyangiitis|Granulomatosis with polyangiitis]] ([[Granulomatosis with polyangiitis|Wegener's]])}}
 {{Family tree| | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | |}}
 {{Family tree| | | | | | | | | | | | | | |,|-|-|-|-|-|-|-|-|-|(| | | | | | | | | | | | |}}
 {{Family tree| | | | | | | | | | | | | | F01 | | | | | | | | F02 | | | | | | | | | | | | | | F01=Known cause|F02=Idiopathic (Unknown)}}
-{{Family tree| |,|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|-|-|-|-|.| | |!| | | | | | | | | | | | | | | | | | | }}
+{{Family tree| |,|-|-|-|v|-|-|-|v|-|-|-|-|^|-|-|-|-|-|-|.| | |!| | | | | | | | | | | | | | | | | | | }}
-{{Family tree| G01 | | G06 | | G02 | | G03 | | G04 | | G05 | |!| | | | | | | | | | | | | | | | | | | G01=Drug-induced pulmonary toxicity|G06=Occupational and environmental exposure|G02=Radiation-induced lung injury|G03=Aspiration pneumonia|G05=Residual of acute respiratory distress syndrome|G04=Smoking-related}}
+{{Family tree| G01 | | G06 | | G02 | | G03 | | G04 | | G05 | |!| | | | | | | | | | | | | | | | | | | G01=Drug-induced pulmonary toxicity|G06=Occupational and environmental exposure|G02=[[Interstitial lung disease#Radiation-induced lung injury|Radiation-induced lung injury]]|G03=[[Aspiration pneumonia]]|G05=Residual of [[acute respiratory distress syndrome]]|G04=Smoking-related}}
 {{Family tree| | | | | |!| | | | | | | | | | | |!| | | | | | |!| | | | | | | | | | | | | | | | | | | }}
 {{Family tree| |,|-|-|-|+|-|-|-|.| | | |,|-|-|-|+|-|-|-|.| | |!| | | | | | | | | | | | | | | | | | | }}
@@ Line 38: / Line 39: @@
 {{Family tree| | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | }}
 {{Family tree| |,|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|v|-|-|^|v|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|.|}}
-{{Family tree| H01 | | H07 | | H02 | | H08 | | H10 | | H09 | | H11 | | H06 | | H03 | | H04 | | H05 |H07=Idiopathic interstitial pneumonias|H01=Pulmonary alveolar proteinosis|H02=Lymphocytic infiltrative disorders<br>(lymphocytic interstitial pneumonitis<br>associated with connective tissue disease)|H08=Connective tissue<br>diseases|H03=Eosinophilic<br>pneumonias|H09=Inherited diseases|H04=Lymphangioleiomyomatosis|H10=Gastrointestinal or<br>liver diseases|H05=Amyloidosis|H11=Graft-versus-host disease|H06=Pulmonary hemorrhage syndromes}}
+{{Family tree| H01 | | H07 | | H02 | | H08 | | H10 | | H09 | | H11 | | H06 | | H03 | | H04 | | H05 |H07=Idiopathic [[interstitial pneumonias]]|H01=[[Pulmonary alveolar proteinosis]]|H02=Lymphocytic infiltrative disorders<br>(lymphocytic [[interstitial pneumonitis]]<br>associated with connective tissue disease)|H08=Connective tissue<br>diseases|H03=[[Eosinophilic pneumonia|Eosinophilic<br>pneumonias]]|H09=Inherited diseases|H04=[[Lymphangioleiomyomatosis]]|H10=Gastrointestinal or<br>liver diseases|H05=[[Amyloidosis]]|H11=Graft-versus-host disease|H06=Pulmonary hemorrhage syndromes}}
 {{Family tree| | | | | |!| | | | | | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | | }}
 {{Family tree| |,|-|-|-|+|-|-|-|.| | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | | }}
-{{Family tree| I04 | | I05 | | I06 | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | | |I04=Major idiopathic interstitial pneumonias|I05=Rare idiopathic interstitial pneumonias|I06=Unclassifiable idiopathic interstitial pneumonias}}
+{{Family tree| I04 | | I05 | | I06 | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | | |I04=Major idiopathic [[interstitial pneumonias]]|I05=Rare idiopathic [[interstitial pneumonias]]|I06=Unclassifiable idiopathic interstitial pneumonias}}
 {{Family tree| |!| | | |!| | | | | | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | |}}
 {{Family tree| |!| | | |!| | | | | | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | | }}
-{{Family tree|boxstyle=text-align: left; | J01 | | J02 | | | | | | J07 | | J09 | | J08 | | J10 | | J11 | | | | | | | | |J01=• Idiopathic pulmonary fibrosis<br>• Idiopathic nonspecific interstitial pneumonia<br>• Respiratory bronchiolitis-interstitial lung disease<br>• Desquamative interstitial pneumonia<br>• Cryptogenic organising pneumonia<br>• Acute interstitial pneumonia<br>|J02=• Idiopathic lymphoid interstitial pneumonia<br>• Idiopathic pleuroparenchymal fibroelastosis<br>|J07=• Systemic lupus erythematosus<br>• Rheumatoid arthritis<br>• Ankylosing spondylitis<br>• Systemic sclerosis<br>• Sjögren syndrome<br>• Polymyositis<br>• Dermatomyositis<br>|J08=• Tuberous sclerosis<br>• Neurofibromatosis<br>• Niemann-Pick disease<br>• Gaucher disease<br>• Hermansky-Pudlak syndrome<br>|J09=• Crohn disease<br>• Primary biliary cirrhosis<br>• Chronic active hepatitis<br>• Ulcerative colitis<br>|J10=• Bone marrow transplantation<br>• Solid organ transplantation<br>|J11=• Goodpasture syndrome<br>• Idiopathic pulmonary hemosiderosis<br>• Isolated pulmonary capillaritis
+{{Family tree|boxstyle=text-align: left; | J01 | | J02 | | | | | | J07 | | J09 | | J08 | | J10 | | J11 | | | | | | | | |J01=• Idiopathic pulmonary fibrosis<br>• Idiopathic nonspecific interstitial pneumonia<br>• Respiratory bronchiolitis-interstitial lung disease<br>• Desquamative [[interstitial pneumonia]]<br>• [[Cryptogenic organising pneumonia]]<br>• Acute interstitial pneumonia<br>|J02=• Idiopathic lymphoid interstitial pneumonia<br>• Idiopathic pleuroparenchymal fibroelastosis<br>|J07=• [[Systemic lupus erythematosus]]<br>• [[Rheumatoid arthritis]]<br>• [[Ankylosing spondylitis]]<br>• [[Systemic sclerosis]]<br>• [[Sjögren syndrome]]<br>• [[Polymyositis]]<br>• [[Dermatomyositis]]<br>|J08=• [[Tuberous sclerosis]]<br>• [[Neurofibromatosis]]<br>• [[Niemann-Pick disease]]<br>• [[Gaucher disease]]<br>• Hermansky-Pudlak syndrome<br>|J09=• [[Crohn disease]]<br>• [[Primary biliary cirrhosis]]<br>• Chronic active [[hepatitis]]<br>• [[Ulcerative colitis]]<br>|J10=• [[Bone marrow transplantation]]<br>• Solid organ transplantation<br>|J11=• [[Goodpasture syndrome]]<br>• Idiopathic pulmonary hemosiderosis<br>• Isolated pulmonary capillaritis
 |}}
 {{Family tree| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | }}
@@ Line 52: / Line 53: @@
 {{Family tree/end}}
 </small>
+</div>
 ==Pathophysiology==
+*Interstitial lung disease is a group of disorders affecting pulmonary [[parenchyma]].
+*The exact pathogenesis of these disorders is not completely understood.
+*There are multiple initiating factors that may cause [[pulmonary]] injury. However, immunopathogenic responses of [[lung]] tissue are quite similar.
+*There are two major histopathologic patterns in response to [[lung]] injury which include:
+**[[Inflammation]] and [[fibrosis]] pattern
+**[[Granulomatous]] pattern
+<br>
 <div style="text-align: center;">'''Algorithm showing pathophysiology of Interstitial Lung Disease'''<ref name="pmid25726561">{{cite journal| author=Bagnato G, Harari S| title=Cellular interactions in the pathogenesis of interstitial lung diseases. | journal=Eur Respir Rev | year= 2015 | volume= 24 | issue= 135 | pages= 102-14 | pmid=25726561 | doi=10.1183/09059180.00003214 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25726561  }} </ref></div>
@@ Line 62: / Line 71: @@
 {{Family tree| | | | | | | | | | | | | | | | | S01 | | | | | | | | | | S02 | | | | | | | S01=[[Parenchymal]] injury|S02=[[Vascular]] injury}}
 {{Family tree| | | | | | | | | | | | | | | | | |!| | | | | | | | | | | |!| | | | | | | | }}
-{{Family tree| | | | | | | | R01 | | | | | | | |!| | | | | | | | | | | R02 | | | | | | | R01=[[Mast cell]]s in [[lungs]] in response to tissue injury|R02= LPA6, LPA2, and LPA4 receptors }}
+{{Family tree| | | | | | | | R01 | | | | | | | |!| | | | | | | | | | | R02 | | | | | | | R01=[[Mast cell]]s in [[lungs]] in response to tissue injury|R02= LPA6, LPA2, and LPA4 receptors<ref name="pmid23084965">{{cite journal| author=Ren Y, Guo L, Tang X, Apparsundaram S, Kitson C, Deguzman J et al.| title=Comparing the differential effects of LPA on the barrier function of human pulmonary endothelial cells. | journal=Microvasc Res | year= 2013 | volume= 85 | issue=  | pages= 59-67 | pmid=23084965 | doi=10.1016/j.mvr.2012.10.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23084965  }} </ref>}}
 {{Family tree| | | | | | |,|-|^|-|.| | | | | | |!| | | | | | | | | |,|-|^|-|.| | | | | | }}
-{{Family tree| | Q01 | | Q06 | | Q03 | | | | | Q02 | | | | | | | | Q04 | | Q05 | | | | | Q01=Decreased [[Secreted frizzled-related protein 1|sFRP-1 (secreted frizzled-related protein 1)]] in [[fibroblasts]]|Q02= [[Insulin-like growth factor]] ([[IGF]]) signalling<ref name="pmid21360508">{{cite journal| author=Hsu E, Shi H, Jordan RM, Lyons-Weiler J, Pilewski JM, Feghali-Bostwick CA| title=Lung tissues in patients with systemic sclerosis have gene expression patterns unique to pulmonary fibrosis and pulmonary hypertension. | journal=Arthritis Rheum | year= 2011 | volume= 63 | issue= 3 | pages= 783-94 | pmid=21360508 | doi=10.1002/art.30159 | pmc=3139818 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21360508  }} </ref>|Q03=[[Transforming growth factor-β]] ([[TGF-β]])|Q04= Reduced expression of angiogenic factors,<br>[[vascular endothelial growth factor]] (VEGF)|Q05=Elevation of angiostatic factors,<br>pigment epithelium-derived factor|Q06=Secretes [[tryptase]]}}
+{{Family tree| | Q01 | | Q06 | | Q03 | | | | | Q02 | | | | | | | | Q04 | | Q05 | | | | | Q01=Decreased [[Secreted frizzled-related protein 1|sFRP-1 (secreted frizzled-related protein 1)]] in [[fibroblasts]]<ref name="pmid21360508">{{cite journal| author=Hsu E, Shi H, Jordan RM, Lyons-Weiler J, Pilewski JM, Feghali-Bostwick CA| title=Lung tissues in patients with systemic sclerosis have gene expression patterns unique to pulmonary fibrosis and pulmonary hypertension. | journal=Arthritis Rheum | year= 2011 | volume= 63 | issue= 3 | pages= 783-94 | pmid=21360508 | doi=10.1002/art.30159 | pmc=3139818 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21360508  }} </ref>|Q02= [[Insulin-like growth factor]] ([[IGF]]) signalling<ref name="pmid21360508">{{cite journal| author=Hsu E, Shi H, Jordan RM, Lyons-Weiler J, Pilewski JM, Feghali-Bostwick CA| title=Lung tissues in patients with systemic sclerosis have gene expression patterns unique to pulmonary fibrosis and pulmonary hypertension. | journal=Arthritis Rheum | year= 2011 | volume= 63 | issue= 3 | pages= 783-94 | pmid=21360508 | doi=10.1002/art.30159 | pmc=3139818 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21360508  }} </ref>|Q03=[[Transforming growth factor-β]] ([[TGF-β]])<ref name="pmid19926870">{{cite journal| author=Andersson CK, Mori M, Bjermer L, Löfdahl CG, Erjefält JS| title=Alterations in lung mast cell populations in patients with chronic obstructive pulmonary disease. | journal=Am J Respir Crit Care Med | year= 2010 | volume= 181 | issue= 3 | pages= 206-17 | pmid=19926870 | doi=10.1164/rccm.200906-0932OC | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19926870  }} </ref>|Q04= Reduced expression of angiogenic factors,<br>[[vascular endothelial growth factor]] (VEGF)<ref name="pmid14754760">{{cite journal| author=Ebina M, Shimizukawa M, Shibata N, Kimura Y, Suzuki T, Endo M et al.| title=Heterogeneous increase in CD34-positive alveolar capillaries in idiopathic pulmonary fibrosis. | journal=Am J Respir Crit Care Med | year= 2004 | volume= 169 | issue= 11 | pages= 1203-8 | pmid=14754760 | doi=10.1164/rccm.200308-1111OC | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14754760  }} </ref>|Q05=Elevation of angiostatic factors,<br>pigment epithelium-derived factor<ref name="pmid15117744">{{cite journal| author=Cosgrove GP, Brown KK, Schiemann WP, Serls AE, Parr JE, Geraci MW et al.| title=Pigment epithelium-derived factor in idiopathic pulmonary fibrosis: a role in aberrant angiogenesis. | journal=Am J Respir Crit Care Med | year= 2004 | volume= 170 | issue= 3 | pages= 242-51 | pmid=15117744 | doi=10.1164/rccm.200308-1151OC | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15117744  }} </ref>|Q06=Secretes [[tryptase]]}}
 {{Family tree| | |!| | | |!| | | |!| | | |,|-|-|^|-|-|.| | | | | | |`|-|v|-|'| | | | | | }}
-{{Family tree| | P01 | | P02 | | P05 | | P04 | | | | P03 |.| | | | | | P06 | | | | | P01=[[Wnt signaling pathway|Wnt/β-catenin signalling pathway]]|P02=PAR-2/protein kinase (PK)C-α/Raf-1/p44/42 signaling pathway|P03=[[IGFBP3|IGF-binding protein 3 (IGFBP-3]])|P04=[[Insulin-like growth factor binding protein|IGF-binding protein 5 (IGFBP-5)]]<ref name="pmid21511034">{{cite journal| author=Bhattacharyya S, Wu M, Fang F, Tourtellotte W, Feghali-Bostwick C, Varga J| title=Early growth response transcription factors: key mediators of fibrosis and novel targets for anti-fibrotic therapy. | journal=Matrix Biol | year= 2011 | volume= 30 | issue= 4 | pages= 235-42 | pmid=21511034 | doi=10.1016/j.matbio.2011.03.005 | pmc=3135176 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21511034  }} </ref>|P05=Upregulation of Egr-1 (early growth response protein 1)<ref name="pmid19628764">{{cite journal| author=Yasuoka H, Hsu E, Ruiz XD, Steinman RA, Choi AM, Feghali-Bostwick CA| title=The fibrotic phenotype induced by IGFBP-5 is regulated by MAPK activation and egr-1-dependent and -independent mechanisms. | journal=Am J Pathol | year= 2009 | volume= 175 | issue= 2 | pages= 605-15 | pmid=19628764 | doi=10.2353/ajpath.2009.080991 | pmc=2716960 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19628764  }} </ref>|P06=Loss of [[endothelial]] barrier function }}
+{{Family tree| | P01 | | P02 | | P05 | | P04 | | | | P03 |.| | | | | | P06 | | | | | P01=[[Wnt signaling pathway|Wnt/β-catenin signalling pathway]]<ref name="pmid18478089">{{cite journal| author=Königshoff M, Balsara N, Pfaff EM, Kramer M, Chrobak I, Seeger W et al.| title=Functional Wnt signaling is increased in idiopathic pulmonary fibrosis. | journal=PLoS One | year= 2008 | volume= 3 | issue= 5 | pages= e2142 | pmid=18478089 | doi=10.1371/journal.pone.0002142 | pmc=2374879 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18478089  }} </ref><ref name="pmid21454805">{{cite journal| author=Lam AP, Flozak AS, Russell S, Wei J, Jain M, Mutlu GM et al.| title=Nuclear β-catenin is increased in systemic sclerosis pulmonary fibrosis and promotes lung fibroblast migration and proliferation. | journal=Am J Respir Cell Mol Biol | year= 2011 | volume= 45 | issue= 5 | pages= 915-22 | pmid=21454805 | doi=10.1165/rcmb.2010-0113OC | pmc=3262680 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21454805  }} </ref>|P02=PAR-2/protein kinase (PK)C-α/Raf-1/p44/42 signaling pathway<ref name="pmid23562441">{{cite journal| author=Wygrecka M, Dahal BK, Kosanovic D, Petersen F, Taborski B, von Gerlach S et al.| title=Mast cells and fibroblasts work in concert to aggravate pulmonary fibrosis: role of transmembrane SCF and the PAR-2/PKC-α/Raf-1/p44/42 signaling pathway. | journal=Am J Pathol | year= 2013 | volume= 182 | issue= 6 | pages= 2094-108 | pmid=23562441 | doi=10.1016/j.ajpath.2013.02.013 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23562441  }} </ref>|P03=[[IGFBP3|IGF-binding protein 3 (IGFBP-3]])|P04=[[Insulin-like growth factor binding protein|IGF-binding protein 5 (IGFBP-5)]]<ref name="pmid21511034">{{cite journal| author=Bhattacharyya S, Wu M, Fang F, Tourtellotte W, Feghali-Bostwick C, Varga J| title=Early growth response transcription factors: key mediators of fibrosis and novel targets for anti-fibrotic therapy. | journal=Matrix Biol | year= 2011 | volume= 30 | issue= 4 | pages= 235-42 | pmid=21511034 | doi=10.1016/j.matbio.2011.03.005 | pmc=3135176 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21511034  }} </ref>|P05=Upregulation of Egr-1 (early growth response protein 1)<ref name="pmid19628764">{{cite journal| author=Yasuoka H, Hsu E, Ruiz XD, Steinman RA, Choi AM, Feghali-Bostwick CA| title=The fibrotic phenotype induced by IGFBP-5 is regulated by MAPK activation and egr-1-dependent and -independent mechanisms. | journal=Am J Pathol | year= 2009 | volume= 175 | issue= 2 | pages= 605-15 | pmid=19628764 | doi=10.2353/ajpath.2009.080991 | pmc=2716960 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19628764  }} </ref>|P06=Loss of [[endothelial]] barrier function }}
 {{Family tree| |!| |!| | |!| | | |!| | | |!| | | | | |!| |!| | | | | | |!| | | | | | | | }}
-{{Family tree| O01 |!| | |!| | | |`|-|-|-|^|.| | | | |!| O02 | | | | | |!| | | | | | | | O01=Dysregulation of repair in [[lung tissue]]  and activation of [[fibroblasts]]|O02=Regulates [[transforming growth factor-β]] ([[TGF-β]]) }}
+{{Family tree| O01 |!| | |!| | | |`|-|-|-|^|.| | | | |!| O02 | | | | | |!| | | | | | | | O01=Dysregulation of repair in [[lung tissue]]  and activation of [[fibroblasts]]<ref name="pmid23881674">{{cite journal| author=Sun Z, Gong X, Zhu H, Wang C, Xu X, Cui D et al.| title=Inhibition of Wnt/β-catenin signaling promotes engraftment of mesenchymal stem cells to repair lung injury. | journal=J Cell Physiol | year= 2014 | volume= 229 | issue= 2 | pages= 213-24 | pmid=23881674 | doi=10.1002/jcp.24436 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23881674  }} </ref>|O02=Regulates [[transforming growth factor-β]] ([[TGF-β]]) }}
 {{Family tree| | | |!| | |!| | | | | | | | |!| | | | |!|,|'| | | | | | |!| | | | | | | | }}
 {{Family tree| | | |!| | |!| | | | | | | | |!| | | | A02 | | | | | | | |!| | | | | | | | A01=|A02=Induction of [[syndecan-2|syndecan-2 (SDC2)]]<ref name="pmid22900087">{{cite journal| author=Ruiz XD, Mlakar LR, Yamaguchi Y, Su Y, Larregina AT, Pilewski JM et al.| title=Syndecan-2 is a novel target of insulin-like growth factor binding protein-3 and is over-expressed in fibrosis. | journal=PLoS One | year= 2012 | volume= 7 | issue= 8 | pages= e43049 | pmid=22900087 | doi=10.1371/journal.pone.0043049 | pmc=3416749 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22900087  }} </ref>}}
@@ Line 79: / Line 88: @@
 {{Family tree| | | | | | | | | | | | D01 | |!| | D02 | | | | | | | | | |!| | | | | | | | D01=Altered PTEN (phosphatase and tensin homologue)/Akt axis|D02=Acquire contractile stress fibres}}
 {{Family tree| | | | | | | | | | | | |!| | |!| | |!| | | | | | | | | | |!| | | | | | | | }}
-{{Family tree| | | | | | | | | | | | G01 | |!| | G02 | | G03 | | | | | |!| | | | | | | | G01= Inactivates [[FOX proteins|Fox (forkhead box) O3a]]<ref name="pmid23580232">{{cite journal| author=Nho RS, Peterson M, Hergert P, Henke CA| title=FoxO3a (Forkhead Box O3a) deficiency protects Idiopathic Pulmonary Fibrosis (IPF) fibroblasts from type I polymerized collagen matrix-induced apoptosis via caveolin-1 (cav-1) and Fas. | journal=PLoS One | year= 2013 | volume= 8 | issue= 4 | pages= e61017 | pmid=23580232 | doi=10.1371/journal.pone.0061017 | pmc=3620276 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23580232  }} </ref>|G02=Protomyofibroblast, composed of cytoplasmic [[actins]]|G03=[[Pleural]] [[mesothelial]] cells (PMCs)}}
+{{Family tree| | | | | | | | | | | | G01 | |!| | G02 | | G03 | | | | | |!| | | | | | | | G01= Inactivates [[FOX proteins|Fox (forkhead box) O3a]]<ref name="pmid23580232">{{cite journal| author=Nho RS, Peterson M, Hergert P, Henke CA| title=FoxO3a (Forkhead Box O3a) deficiency protects Idiopathic Pulmonary Fibrosis (IPF) fibroblasts from type I polymerized collagen matrix-induced apoptosis via caveolin-1 (cav-1) and Fas. | journal=PLoS One | year= 2013 | volume= 8 | issue= 4 | pages= e61017 | pmid=23580232 | doi=10.1371/journal.pone.0061017 | pmc=3620276 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23580232  }} </ref>|G02=Protomyofibroblast, composed of cytoplasmic [[actins]]|G03=[[Pleural]] [[mesothelial]] cells (PMCs)<ref name="pmid21737551">{{cite journal| author=Mubarak KK, Montes-Worboys A, Regev D, Nasreen N, Mohammed KA, Faruqi I et al.| title=Parenchymal trafficking of pleural mesothelial cells in idiopathic pulmonary fibrosis. | journal=Eur Respir J | year= 2012 | volume= 39 | issue= 1 | pages= 133-40 | pmid=21737551 | doi=10.1183/09031936.00141010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21737551  }} </ref><ref name="pmid19411308">{{cite journal| author=Nasreen N, Mohammed KA, Mubarak KK, Baz MA, Akindipe OA, Fernandez-Bussy S et al.| title=Pleural mesothelial cell transformation into myofibroblasts and haptotactic migration in response to TGF-beta1 in vitro. | journal=Am J Physiol Lung Cell Mol Physiol | year= 2009 | volume= 297 | issue= 1 | pages= L115-24 | pmid=19411308 | doi=10.1152/ajplung.90587.2008 | pmc=2711818 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19411308  }} </ref>}}
 {{Family tree| | | | | | | | | | | | |!| | |!| | |!| | | |!| | | | | | |!| | | | | | | | }}
 {{Family tree| | | | | | | | | | | | H01 | |!| | H02 | | H03 | | | | | |!| | | | | | | | H01= Downregulation of [[Caveolin 1|caveolin-1 (cav-1)]] and Fas expression<ref name="pmid18759267">{{cite journal| author=Del Galdo F, Sotgia F, de Almeida CJ, Jasmin JF, Musick M, Lisanti MP et al.| title=Decreased expression of caveolin 1 in patients with systemic sclerosis: crucial role in the pathogenesis of tissue fibrosis. | journal=Arthritis Rheum | year= 2008 | volume= 58 | issue= 9 | pages= 2854-65 | pmid=18759267 | doi=10.1002/art.23791 | pmc=2770094 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18759267  }} </ref>|H02=De novo expression of α-smooth muscle actin (α-SMA)|H03=TGF-β1-dependent mesothelial–mesenchymal transition}}
@@ Line 88: / Line 97: @@
 {{Family tree| | | | | | | | | | | | | | | |!| | | |!| J02 | | | J03 | |!| | | | | | | | J02=Different ranges of contractions mediated by RhoA/Rho-associated kinase|J03=Changes in intracellular calcium concentrations}}
 {{Family tree| | | | | | | | | | | | | | | |!| | | |!| | |`|-|v|-|'| | |!| | | | | | | | }}
-{{Family tree| | | | | | | | K01 | | | | | |!| |,|-|'| | | | K02 | | | |!| | | | | K03 | K01=Recruitement of fibrocytes in [[lungs]]|K02=Lock step mechanism of cyclic and contractile events<ref name="pmid20427321">{{cite journal| author=Castella LF, Buscemi L, Godbout C, Meister JJ, Hinz B| title=A new lock-step mechanism of matrix remodelling based on subcellular contractile events. | journal=J Cell Sci | year= 2010 | volume= 123 | issue= Pt 10 | pages= 1751-60 | pmid=20427321 | doi=10.1242/jcs.066795 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20427321  }} </ref>|K03=[[T helper cell|T-helper cell type 2]] on site of injury}}
+{{Family tree| | | | | | | | K01 | | | | | |!| |,|-|'| | | | K02 | | | |!| | | | | K03 | K01=Recruitement of fibrocytes in [[lungs]]|K02=Lock step mechanism of cyclic and contractile events<ref name="pmid20427321">{{cite journal| author=Castella LF, Buscemi L, Godbout C, Meister JJ, Hinz B| title=A new lock-step mechanism of matrix remodelling based on subcellular contractile events. | journal=J Cell Sci | year= 2010 | volume= 123 | issue= Pt 10 | pages= 1751-60 | pmid=20427321 | doi=10.1242/jcs.066795 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20427321  }} </ref>|K03=[[T helper cell|T-helper cell type 2]] on site of injury<ref name="pmid15802346">{{cite journal| author=Capelli A, Di Stefano A, Gnemmi I, Donner CF| title=CCR5 expression and CC chemokine levels in idiopathic pulmonary fibrosis. | journal=Eur Respir J | year= 2005 | volume= 25 | issue= 4 | pages= 701-7 | pmid=15802346 | doi=10.1183/09031936.05.00082604 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15802346  }} </ref><ref name="pmid15383605">{{cite journal| author=Belperio JA, Dy M, Murray L, Burdick MD, Xue YY, Strieter RM et al.| title=The role of the Th2 CC chemokine ligand CCL17 in pulmonary fibrosis. | journal=J Immunol | year= 2004 | volume= 173 | issue= 7 | pages= 4692-8 | pmid=15383605 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15383605  }} </ref>}}
 {{Family tree| | | | | | | | |!| | | | | | |!| |!| | | | | | |!| | | | |!| | | | | |!| | }}
-{{Family tree| | | | | | | | L01 | | | | | | L02 | | | | | | L03 | | | |!| | | | | L04 | |L01=Upregulation of [[CXCR4|C-X-C chemokine receptor type 4 (CXCR4)]]<br>on [[fibrocytes]] and its ligand<br>CXCL12 (stromal cell-derived factor 1)|L02=Excess extracellular matrix production|L03=Exerting traction force|L04=[[Interleukin-13]]}}
+{{Family tree| | | | | | | | L01 | | | | | | L02 | | | | | | L03 | | | |!| | | | | L04 | |L01=Upregulation of [[CXCR4|C-X-C chemokine receptor type 4 (CXCR4)]]<br>on [[fibrocytes]] and its ligand<br>CXCL12 (stromal cell-derived factor 1)<ref name="pmid18374622">{{cite journal| author=Andersson-Sjöland A, de Alba CG, Nihlberg K, Becerril C, Ramírez R, Pardo A et al.| title=Fibrocytes are a potential source of lung fibroblasts in idiopathic pulmonary fibrosis. | journal=Int J Biochem Cell Biol | year= 2008 | volume= 40 | issue= 10 | pages= 2129-40 | pmid=18374622 | doi=10.1016/j.biocel.2008.02.012 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18374622  }} </ref>|L02=Excess extracellular matrix production|L03=Exerting traction force|L04=[[Interleukin-13]]}}
 {{Family tree| | | | | | | | |!| | | | | | | |!|,|-|-|-|-|-|-|'| | | | |!| | | | | |!| | }}
-{{Family tree| | | | | | | | M01 | | | | | | M02 | | | | | | | | | | | |!| | | | | M03 | |M01=Migration of fibrocytes to the site of injury|M02=Tissue remodelling<ref name="pmid17525249">{{cite journal| author=Hinz B, Phan SH, Thannickal VJ, Galli A, Bochaton-Piallat ML, Gabbiani G| title=The myofibroblast: one function, multiple origins. | journal=Am J Pathol | year= 2007 | volume= 170 | issue= 6 | pages= 1807-16 | pmid=17525249 | doi=10.2353/ajpath.2007.070112 | pmc=1899462 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17525249  }} </ref>|M03=Alternate pathway activation of [[macrophages]]}}
+{{Family tree| | | | | | | | M01 | | | | | | M02 | | | | | | | | | | | |!| | | | | M03 | |M01=Migration of fibrocytes to the site of injury<ref name="pmid15743780">{{cite journal| author=Moore BB, Kolodsick JE, Thannickal VJ, Cooke K, Moore TA, Hogaboam C et al.| title=CCR2-mediated recruitment of fibrocytes to the alveolar space after fibrotic injury. | journal=Am J Pathol | year= 2005 | volume= 166 | issue= 3 | pages= 675-84 | pmid=15743780 | doi=10.1016/S0002-9440(10)62289-4 | pmc=1780139 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15743780  }} </ref>|M02=Tissue remodelling<ref name="pmid17525249">{{cite journal| author=Hinz B, Phan SH, Thannickal VJ, Galli A, Bochaton-Piallat ML, Gabbiani G| title=The myofibroblast: one function, multiple origins. | journal=Am J Pathol | year= 2007 | volume= 170 | issue= 6 | pages= 1807-16 | pmid=17525249 | doi=10.2353/ajpath.2007.070112 | pmc=1899462 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17525249  }} </ref>|M03=Alternate pathway activation of [[macrophages]]<ref name="pmid7995399">{{cite journal| author=Lohmann-Matthes ML, Steinmüller C, Franke-Ullmann G| title=Pulmonary macrophages. | journal=Eur Respir J | year= 1994 | volume= 7 | issue= 9 | pages= 1678-89 | pmid=7995399 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7995399  }} </ref>}}
 {{Family tree| | | | | | | | |!| | | | | | | |!| | | | | | | | | | | | |!| | | | | |!| | }}
 {{Family tree| | | | | | | | |`|-|-|-|-|-|-|.|!|,|-|-|-|-|-|-|-|-|-|-|-|'| | | | | |!| | }}
@@ Line 98: / Line 107: @@
 {{Family tree/end}}
-==References==
+==Differentiating Interstitial Lung Disease from other Diseases==
-{{Respiratory pathology}}
+'''''To review the complete differential diagnosis of dyspnea, [[Dyspnea differential diagnosis|click here.]]'''''
+'''''To review the complete differential diagnosis of hemoptysis, [[Hemoptysis differential diagnosis|click here.]]'''''
+'''''To review the complete differential diagnosis of restrictive lung disease, [[Restrictive lung disease|click here.]]'''''
+<small>
+'''Abbreviations''': '''ABG''': Arterial blood gas, '''BAL''': Bronchoalveolar lavage, '''ESR''': Erythrocyte sedimentation rate, '''CRP''': C–reactive protein, '''FVC''': Forced vital capacity, '''RV''': Residual volume, '''FEV1''': Forced expiratory volume during the 1st second, '''DLCO''': Diffusing capacity of the lungs for carbon monoxide, '''O2''': Oxygen, '''TLC''': Total lung capacity, '''PaO2''': Arterial partial pressure of oxygen, '''FiO2''': Fraction of inspired oxygen, '''LDH''': Lactate dehydrogenase, '''CEA''': Carcinoembryonic antigen, '''Anti-GBM antibody''': Anti-glomerular basement membrane antibody, '''A−a gradient''': Alveolar-arterial gradient, '''PAS''': Periodic acid-Schiff stain, '''LAM''': Lymphangiomyomatosis, '''IgE''': Immunoglobulin E, '''ANCA''': Anti-neutrophil cytoplasmic antibody, '''RBC''': Red blood cell, '''ACE''': Angiotensin-converting enzyme
+<small><small>
+{| class="wikitable"
+! colspan="2" rowspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
+! colspan="15" style="background:#4479BA; color: #FFFFFF;" align="center" |Clinical manifestation
+! colspan="6" style="background:#4479BA; color: #FFFFFF;" align="center" |Investigations
+|-
+! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |History
+! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Symptoms
+! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Physical examination
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab findings
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging
+! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Pulmonary function test
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Bronchoscopy and BAL
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Gold standard
+|-
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Duration
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Age
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Gender
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Family history
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Smoking history
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Environmental exposure
+! style="background:#4479BA; color: #FFFFFF;" align="center" |HIV
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cough
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Wheezing
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Chest pain
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Tachypnea
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Auscultation
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cyanosis
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Clubbing
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Spirometry
+! style="background:#4479BA; color: #FFFFFF;" align="center" |ABG
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |[[Idiopathic pulmonary fibrosis]]<ref name="PolettiRavaglia2013">{{cite journal|last1=Poletti|first1=Venerino|last2=Ravaglia|first2=Claudia|last3=Buccioli|first3=Matteo|last4=Tantalocco|first4=Paola|last5=Piciucchi|first5=Sara|last6=Dubini|first6=Alessandra|last7=Carloni|first7=Angelo|last8=Chilosi|first8=Marco|last9=Tomassetti|first9=Sara|title=Idiopathic Pulmonary Fibrosis: Diagnosis and Prognostic Evaluation|journal=Respiration|volume=86|issue=1|year=2013|pages=5–12|issn=1423-0356|doi=10.1159/000353580}}</ref>
+| align="center" |Chronic
+| align="center" |60−70 years old
+| align="center" |Men
+| align="center" | +
+| align="center" | +
+| align="center" |±
+| align="center" | −
+| align="center" | +
+| align="center" |Dry
+| align="center" | +
+| align="center" | +
+| align="center" | +
+|
+* Inspiratory high−pitched [[rhonchi]]
+* Bibasilar inspiratory [[Rales|crackles]]
+| align="center" | −
+| align="center" | +
+|
+* [[Anti-nuclear antibody|Antinuclear antibody]] +
+* [[Rheumatoid factor]] +
+* Elevated [[Erythrocyte sedimentation rate|ESR]]
+* Elevated [[C-reactive protein|CRP]]
+* [[Polycythemia]]
+|
+* Bibasilar, peripheral reticular abnormalities
+* Focal honeycomb cyst formation
+* Traction [[bronchiectasis]]
+|
+* ↓ [[Vital capacity|FVC]]
+* ↑ [[Residual volume|RV]]
+* Normal [[FEV1/FVC ratio|FEV1/FVC]]
+* ↓ [[DLCO]]
+|
+* ↓ [[Oxygen|O2]]
+|
+* Not required
+* ↑ [[Neutrophil|Neutrophils]]
+* ↑ [[Eosinophil granulocyte|Eosinophils]]
+|
+* Diagnosis of exclusion
+* Lung [[biopsy]]
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" s |Idiopathic nonspecific interstitial pneumonia<ref name="TravisHunninghake2008">{{cite journal|last1=Travis|first1=William D.|last2=Hunninghake|first2=Gary|last3=King|first3=Talmadge E.|last4=Lynch|first4=David A.|last5=Colby|first5=Thomas V.|last6=Galvin|first6=Jeffrey R.|last7=Brown|first7=Kevin K.|last8=Chung|first8=Man Pyo|last9=Cordier|first9=Jean-François|last10=du Bois|first10=Roland M.|last11=Flaherty|first11=Kevin R.|last12=Franks|first12=Teri J.|last13=Hansell|first13=David M.|last14=Hartman|first14=Thomas E.|last15=Kazerooni|first15=Ella A.|last16=Kim|first16=Dong Soon|last17=Kitaichi|first17=Masanori|last18=Koyama|first18=Takashi|last19=Martinez|first19=Fernando J.|last20=Nagai|first20=Sonoko|last21=Midthun|first21=David E.|last22=Müller|first22=Nestor L.|last23=Nicholson|first23=Andrew G.|last24=Raghu|first24=Ganesh|last25=Selman|first25=Moisés|last26=Wells|first26=Athol|title=Idiopathic Nonspecific Interstitial Pneumonia|journal=American Journal of Respiratory and Critical Care Medicine|volume=177|issue=12|year=2008|pages=1338–1347|issn=1073-449X|doi=10.1164/rccm.200611-1685OC}}</ref>
+| align="center" |Acute/Chronic
+| align="center" |50−60 years old
+| align="center" |Female
+| align="center" | +
+| align="center" |−
+| align="center" | −
+| align="center" | +
+| align="center" | +
+| align="center" | +
+| align="center" | +
+| align="center" |−
+| align="center" | +
+|
+* Bibasilar [[Rales|crackles]]
+| align="center" | −
+| align="center" |±
+|
+* Normal
+|
+* Bilateral ground−glass opacities
+* Fine reticular infiltrates
+* Traction [[bronchiectasis]]
+* [[Consolidation (medicine)|Consolidation]]
+|
+* Normal to ↑ [[FEV1/FVC ratio|FEV1/FVC]]
+* ↓ [[Vital capacity|FVC]]
+* ↓ [[Total lung capacity|TLC]]
+|
+* ↓ [[Oxygen|O2]]
+|
+* Nonspecific
+|
+* Lung [[biopsy]] and multidisciplinary approach
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |[[Cryptogenic organizing pneumonia]]<ref name="MehrianDoroudinia2017">{{cite journal|last1=Mehrian|first1=P.|last2=Doroudinia|first2=A.|last3=Rashti|first3=A.|last4=Aloosh|first4=O.|last5=Dorudinia|first5=A.|title=High-resolution computed tomography findings in chronic eosinophilic vs. cryptogenic organising pneumonia|journal=The International Journal of Tuberculosis and Lung Disease|volume=21|issue=11|year=2017|pages=1181–1186|issn=1027-3719|doi=10.5588/ijtld.16.0723}}</ref>
+| align="center" |Acute/subacute
+| align="center" |50−60 years old
+| align="center" |Both
+| align="center" |−
+| align="center" |±
+| align="center" |−
+| align="center" |−
+| align="center" | +
+| align="center" |Dry
+| align="center" | −
+| align="center" |−
+| align="center" |−
+|
+* Inspiratory [[Rales|crackles]]
+| align="center" | −
+| align="center" |−
+|
+* [[Leukocytosis]]
+* Elevated [[Erythrocyte sedimentation rate|ESR]]
+* Elevated [[C-reactive protein|CRP]]
+|
+* [[Alveolus|Alveolar]] filling and air bronchograms
+* Bilateral ground−glass opacities
+* Bilateral [[Consolidation (medicine)|consolidation]]
+|
+* ↓ [[Vital capacity|FVC]]
+* Normal [[FEV1/FVC ratio|FEV1/FVC]]
+* ↓ [[DLCO]]
+|
+* ↓ [[Oxygen|O2]]
+|
+* ↑ [[Lymphocyte|Lymphocytes]]
+* ↑ [[Neutrophil|Neutrophils]]
+* ↑ [[Eosinophil granulocyte|Eosinophils]]
+|
+* Lung [[biopsy]]
+|-
+! colspan="2" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
+! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |History
+! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Symptoms
+! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Physical examination
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab findings
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging
+! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Pulmonary function test
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Bronchoscopy and BAL
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Gold standard
+|-
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Duration
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Age
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Gender
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Family history
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Smoking history
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Environmental exposure
+! style="background:#4479BA; color: #FFFFFF;" align="center" |HIV
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cough
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Wheezing
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Chest pain
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Tachypnea
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Auscultation
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cyanosis
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Clubbing
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Spirometry
+! style="background:#4479BA; color: #FFFFFF;" align="center" |ABG
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |Acute [[interstitial pneumonia]] (Hamman−Rich syndrome)<ref name="ParambilMukhopadhyay2012">{{cite journal|last1=Parambil|first1=Joseph|last2=Mukhopadhyay|first2=Sanjay|title=Acute Interstitial Pneumonia (AIP): Relationship to Hamman-Rich Syndrome, Diffuse Alveolar Damage (DAD), and Acute Respiratory Distress Syndrome (ARDS)|journal=Seminars in Respiratory and Critical Care Medicine|volume=33|issue=05|year=2012|pages=476–485|issn=1069-3424|doi=10.1055/s-0032-1325158}}</ref>
+| align="center" |Acute
+| align="center" |50−60 years old
+| align="center" |Both
+| align="center" | −
+| align="center" |−
+| align="center" |−
+| align="center" |−
+| align="center" | +
+| align="center" | +
+| align="center" |−
+| align="center" |−
+| align="center" | +
+|
+* Diffuse [[Rales|crackles]]
+| align="center" | −
+| align="center" | −
+|
+* [[Leukocytosis]]
+|
+* Bilateral and symmetric, diffuse ground glass
+* [[Alveolus|Alveolar]] consolidation opacities
+* Traction [[bronchiectasis]]
+* Honeycomb [[fibrosis]]
+|
+* N/A
+|
+* ↓ [[Oxygen|O2]]
+* [[Pulmonary gas pressures|PaO2]]/[[FiO2]] <200 mmHg
+|
+* Nonspecific
+* ↑ [[Neutrophil|Neutrophils]]
+* ↑ Atypical [[Epithelium|epithelial cells]]
+|
+* Lung [[biopsy]]
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |Lymphocytic [[interstitial pneumonia]]<ref name="PanchabhaiFarver2016">{{cite journal|last1=Panchabhai|first1=Tanmay S.|last2=Farver|first2=Carol|last3=Highland|first3=Kristin B.|title=Lymphocytic Interstitial Pneumonia|journal=Clinics in Chest Medicine|volume=37|issue=3|year=2016|pages=463–474|issn=02725231|doi=10.1016/j.ccm.2016.04.009}}</ref>
+| align="center" |Subacute
+| align="center" |30−40 years old
+| align="center" |Female
+| align="center" |−
+| align="center" |−
+| align="center" |−
+| align="center" |±
+| align="center" | +
+| align="center" | +
+| align="center" | +
+| align="center" | +
+| align="center" | −
+|
+* Diffuse [[Rales|crackles]]
+| align="center" | −
+| align="center" | +
+|
+* [[Gammopathy]]
+|
+* Diffuse ground glass attenuation with [[fibrosis]]
+* Centrilobular and subpleural [[Nodule (medicine)|nodules]]
+* Lung [[Cyst|cysts]]
+|
+* ↓ [[Vital capacity|FVC]]
+* ↓ [[Total lung capacity|TLC]]
+* ↓ [[DLCO]]
+|
+* ↓ [[Oxygen|O2]]
+|
+* Nonspecific
+* ↑ Total [[Bronchoalveolar lavage|BAL]] cell count
+* [[Bronchoalveolar lavage|BAL]] [[lymphocytosis]]
+|
+* Lung [[biopsy]]
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |Respiratory bronchiolitis−interstitial lung disease<ref name="SieminskaKuziemski2014">{{cite journal|last1=Sieminska|first1=Alicja|last2=Kuziemski|first2=Krzysztof|title=Respiratory bronchiolitis-interstitial lung disease|journal=Orphanet Journal of Rare Diseases|volume=9|issue=1|year=2014|issn=1750-1172|doi=10.1186/s13023-014-0106-8}}</ref>
+| align="center" |Subacute
+| align="center" |30−40 years old
+| align="center" |Both
+| align="center" |−
+| align="center" | +
+| align="center" |−
+| align="center" | −
+| align="center" | +
+| align="center" |Dry
+| align="center" | +
+| align="center" | −
+| align="center" | −
+|
+* Inspiratory high−pitched [[rhonchi]]
+* Fine, bibasilar end−inspiratory [[Rales|crackles]]
+| align="center" |−
+| align="center" |−
+|
+* Nonspecific
+|
+* Diffuse or patchy ground glass opacities in a mosaic pattern
+* Fine [[Nodule (medicine)|nodules]]
+* Air trapping
+|
+* ↓ [[Vital capacity|FVC]]
+* ↓ [[Total lung capacity|TLC]]
+* ↓ [[DLCO]]
+|
+* ↓ [[Oxygen|O2]]
+|
+* ↑ [[Macrophage|Macrophages]]
+|
+* Clinical evaluation and investigations
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |Desquamative interstitial pneumonia<ref name="RyuMyers2005">{{cite journal|last1=Ryu|first1=Jay H.|last2=Myers|first2=Jeffrey L.|last3=Capizzi|first3=Stephen A.|last4=Douglas|first4=William W.|last5=Vassallo|first5=Robert|last6=Decker|first6=Paul A.|title=Desquamative Interstitial Pneumonia and Respiratory Bronchiolitis-Associated Interstitial Lung Disease|journal=Chest|volume=127|issue=1|year=2005|pages=178–184|issn=00123692|doi=10.1378/chest.127.1.178}}</ref><ref name="CraigWells2004">{{cite journal|last1=Craig|first1=P J|last2=Wells|first2=A U|last3=Doffman|first3=S|last4=Rassl|first4=D|last5=Colby|first5=T V|last6=Hansell|first6=D M|last7=du Bois|first7=R M|last8=Nicholson|first8=A G|title=Desquamative interstitial pneumonia, respiratory bronchiolitis and their relationship to smoking|journal=Histopathology|volume=45|issue=3|year=2004|pages=275–282|issn=0309-0167|doi=10.1111/j.1365-2559.2004.01921.x}}</ref>
+| align="center" |Chronic
+| align="center" |40−50 years old
+| align="center" |Both
+| align="center" |−
+| align="center" | +
+| align="center" |−
+| align="center" |−
+| align="center" | +
+| align="center" |Dry
+| align="center" | +
+| align="center" |−
+| align="center" |−
+|
+* Fine, bibasilar end−inspiratory [[Rales|crackles]]
+| align="center" |−
+| align="center" |−
+|
+* Nonspecific
+|
+* Ground glass opacities without the peripheral reticular and reticulonodular opacities
+|
+* ↓ [[DLCO]]
+|
+* ↓ [[Oxygen|O2]]
+|
+* ↑ [[Eosinophil granulocyte|Eosinophils]]
+|
+* Lung [[biopsy]]
+|-
+! colspan="2" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
+! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |History
+! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Symptoms
+! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Physical examination
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab findings
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging
+! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Pulmonary function test
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Bronchoscopy and BAL
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Gold standard
+|-
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Duration
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Age
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Gender
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Family history
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Smoking history
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Environmental exposure
+! style="background:#4479BA; color: #FFFFFF;" align="center" |HIV
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cough
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Wheezing
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Chest pain
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Tachypnea
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Auscultation
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cyanosis
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Clubbing
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Spirometry
+! style="background:#4479BA; color: #FFFFFF;" align="center" |ABG
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |Pulmonary [[Langerhans cell granulomatosis]]<ref name="BlakleyDutcher2018">{{cite journal|last1=Blakley|first1=Matthew P.|last2=Dutcher|first2=Janice P.|last3=Wiernik|first3=Peter H.|title=Pulmonary Langerhans cell histiocytosis, acute myeloid leukemia, and myelofibrosis in a large family and review of the literature|journal=Leukemia Research|volume=67|year=2018|pages=39–44|issn=01452126|doi=10.1016/j.leukres.2018.01.011}}</ref>
+| align="center" |Chronic
+| align="center" |20−40 years old
+| align="center" |Both
+| align="center" | +
+| align="center" | +
+| align="center" |−
+| align="center" |−
+| align="center" |±
+| align="center" |Dry
+| align="center" | +
+| align="center" | +
+| align="center" |−
+|
+* Unremarkable
+|−
+|−
+|
+* Nonspecific
+|
+* Mid to upper lung zone [[Cyst|cysts]] and [[Nodule (medicine)|nodules]]
+* Reticular and [[Nodule (medicine)|nodular]] opacities
+* Recurrent spontaneous [[pneumothorax]]
+|
+* ↓ [[FEV1/FVC ratio|FEV1/FVC]]
+|
+* Normal
+|
+* >5 percent [[Langerhans cell|langerhans cells]] (CD−1a positive)
+|
+* Lung [[biopsy]]
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |[[Pulmonary alveolar proteinosis]]<ref name="pmid29493933">{{cite journal |vauthors=Carrington JM, Hershberger DM |title= |journal= |volume= |issue= |pages= |date= |pmid=29493933 |doi= |url=}}</ref><ref name="KianiParsa2018">{{cite journal|last1=Kiani|first1=Arda|last2=Parsa|first2=Tahereh|last3=Adimi Naghan|first3=Parisa|last4=Dutau|first4=Hervé|last5=Razavi|first5=Fatemeh|last6=Farzanegan|first6=Behrooz|last7=Pourabdollah Tootkaboni|first7=Mahsa|last8=Abedini|first8=Atefeh|title=An eleven-year retrospective cross-sectional study on pulmonary alveolar proteinosis|journal=Advances in Respiratory Medicine|volume=86|issue=1|year=2018|pages=7–12|issn=2543-6031|doi=10.5603/ARM.2018.0003}}</ref>
+| align="center" |Acute/chronic
+| align="center" |40−50 years old
+| align="center" |Male
+| align="center" | +
+| align="center" | +
+| align="center" | +
+| align="center" |−
+| align="center" | +
+| align="center" | +
+| align="center" | +
+| align="center" |−
+| align="center" |−
+|
+*  Inspiratory [[Rales|crackles]]
+| align="center" | +
+| align="center" | +
+|
+* ↑ [[Lactate dehydrogenase|LDH]]
+* ↑ [[CEA]]
+* ↑ [[Surfactant]] protein A, B, and D
+* [[Anti-glomerular basement membrane antibody|Anti-GBM antibody]] +
+* [[Polycythemia]]
+* [[Hypergammaglobulinemia]]
+|
+* Bbilateral perihilar and basilar [[Alveolus|alveolar]] opacities without air−bronchograms
+* "Bat wing" distribution
+* Intralobular thickening
+* Diffuse ground−glass opacities
+|
+* ↑ [[FEV1/FVC ratio|FEV1/FVC]]
+* ↑ [[A-a gradient|A−a gradient]]
+* ↓ [[DLCO]]
+|
+* ↓ [[Pulmonary gas pressures|PaO2]]
+* ↓ [[Oxygen|O2]]
+* [[Respiratory alkalosis]]
+|
+* Large foamy [[Macrophage|macrophages]] with amorphous [[Periodic acid-Schiff stain|PAS]]−positive material
+* ↑  [[Lymphocyte|Lymphocytes]]
+|
+* [[Bronchoscopy]] and [[Bronchoalveolar lavage|BAL]]
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |Pulmonary [[lymphangioleiomyomatosis]]<ref name="XuLo2014">{{cite journal|last1=Xu|first1=Kai-Feng|last2=Lo|first2=Bee Hong|title=Lymphangioleiomyomatosis: differential diagnosis and optimal management|journal=Therapeutics and Clinical Risk Management|year=2014|pages=691|issn=1178-203X|doi=10.2147/TCRM.S50784}}</ref>
+| align="center" |Acute/chronic
+| align="center" |30−40 years old
+| align="center" |Female
+| align="center" | +
+| align="center" | +
+| align="center" |−
+| align="center" |−
+| align="center" | +
+| align="center" |Bloody
+| align="center" | +
+| align="center" | +
+| align="center" |−
+|
+* Decreased [[breath sounds]]
+| align="center" |−
+| align="center" | +
+|
+* ↑ [[Vascular endothelial growth factor|Vascular endothelial growth factor−D]] (VEGF−D)
+|
+* [[Pneumothorax]]
+* [[Chylothorax]]
+* Thin−walled round [[Cyst|cystic]] lesions
+|
+* ↓ [[FEV1/FVC ratio|FEV1/FVC]]
+|
+* ↓ [[Pulmonary gas pressures|PaO2]]
+* ↓ [[Oxygen|O2]]
+|
+* [[LAM]] cells +
+|
+* Lung [[biopsy]]
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |[[Eosinophilic pneumonia]]<ref name="BernheimMcLoud2017">{{cite journal|last1=Bernheim|first1=Adam|last2=McLoud|first2=Theresa|title=A Review of Clinical and Imaging Findings in Eosinophilic Lung Diseases|journal=American Journal of Roentgenology|volume=208|issue=5|year=2017|pages=1002–1010|issn=0361-803X|doi=10.2214/AJR.16.17315}}</ref>
+| align="center" |Acute/chronic
+| align="center" |20−40 years old
+| align="center" |Male
+| align="center" |−
+| align="center" |−
+| align="center" |−
+| align="center" |−
+| align="center" | +
+| align="center" |Dry
+| align="center" | +
+| align="center" | +
+| align="center" | +
+|
+* Decreased [[breath sounds]]
+| align="center" |−
+| align="center" |−
+|
+* Neutrophilic [[leukocytosis]]
+* ↑ [[Eosinophil granulocyte|Eosinophils]]
+* Elevated [[Erythrocyte sedimentation rate|ESR]]
+* Elevated [[C-reactive protein|CRP]]
+* Elevated [[Immunoglobulin E|IgE]] level
+|
+* Bilateral diffuse mixed ground glass and reticular opacities
+* Small bilateral [[Pleural effusion|pleural effusions]]
+* Centrilobular [[Nodule (medicine)|nodules]] and air−space [[Consolidation (medicine)|consolidation]]
+|
+* ↑ [[FEV1/FVC ratio|FEV1/FVC]]
+* ↓ [[DLCO]]
+|
+* ↓ [[Pulmonary gas pressures|PaO2]]
+* ↓ [[Oxygen|O2]]
+|
+* [[Eosinophilia]]
+|
+* Clinical evaluation and investigations
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |[[Hypersensitivity pneumonitis]]<ref name="MillerAllen2018">{{cite journal|last1=Miller|first1=Ross|last2=Allen|first2=Timothy Craig|last3=Barrios|first3=Roberto J.|last4=Beasley|first4=Mary Beth|last5=Burke|first5=Louise|last6=Cagle|first6=Philip T.|last7=Capelozzi|first7=Vera Luiza|last8=Ge|first8=Yimin|last9=Hariri|first9=Lida P.|last10=Kerr|first10=Keith M.|last11=Khoor|first11=Andras|last12=Larsen|first12=Brandon T.|last13=Mark|first13=Eugene J.|last14=Matsubara|first14=Osamu|last15=Mehrad|first15=Mitra|last16=Mino-Kenudson|first16=Mari|last17=Raparia|first17=Kirtee|last18=Roden|first18=Anja Christiane|last19=Russell|first19=Prudence|last20=Schneider|first20=Frank|last21=Sholl|first21=Lynette M.|last22=Smith|first22=Maxwell Lawrence|title=Hypersensitivity Pneumonitis A Perspective From Members of the Pulmonary Pathology Society|journal=Archives of Pathology & Laboratory Medicine|volume=142|issue=1|year=2018|pages=120–126|issn=0003-9985|doi=10.5858/arpa.2017-0138-SA}}</ref>
+| align="center" |Acute/subacute/chronic
+| align="center" |40−60 years old
+| align="center" |Both
+| align="center" |−
+| align="center" |±
+| align="center" | +
+| align="center" | −
+| align="center" | +
+| align="center" |Dry/productive
+| align="center" | +
+| align="center" | +
+| align="center" | +
+|
+* Diffuse fine bibasilar [[crackles]]
+| align="center" | −
+| align="center" | +
+|
+* Neutrophilic [[leukocytosis]]
+|
+* Centrilobular ground−glass or nodular opacities of mid−to−upper zone
+* Air−trapping
+|
+* ↓ [[FEV1]]
+* ↓ [[Vital capacity|FVC]]
+|
+* ↓ [[Pulmonary gas pressures|PaO2]]
+* ↓ [[Oxygen|O2]]
+|
+* [[Lymphocytosis]]
+|
+* Lung [[biopsy]]
+|-
+! colspan="2" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
+! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |History
+! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Symptoms
+! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Physical examination
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab findings
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging
+! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Pulmonary function test
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Bronchoscopy and BAL
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Gold standard
+|-
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Duration
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Age
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Gender
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Family history
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Smoking history
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Environmental exposure
+! style="background:#4479BA; color: #FFFFFF;" align="center" |HIV
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cough
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Wheezing
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Chest pain
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Tachypnea
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Auscultation
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cyanosis
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Clubbing
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Spirometry
+! style="background:#4479BA; color: #FFFFFF;" align="center" |ABG
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |[[Occupational lung disease]]<ref name="SirajuddinKanne2009">{{cite journal|last1=Sirajuddin|first1=Arlene|last2=Kanne|first2=Jeffrey P.|title=Occupational Lung Disease|journal=Journal of Thoracic Imaging|volume=24|issue=4|year=2009|pages=310–320|issn=0883-5993|doi=10.1097/RTI.0b013e3181c1a9b3}}</ref>
+| align="center" |Chronic
+| align="center" |Elderly
+| align="center" |Male
+| align="center" | +
+| align="center" | +
+| align="center" | +
+| align="center" |−
+| align="center" |±
+| align="center" | +
+| align="center" | +
+| align="center" | +
+| align="center" | +
+|
+* Fine [[Rales|crackles]]
+| align="center" |Peripheral/central
+| align="center" | +
+|
+* [[Anemia]]
+* [[Neutrophilia]]
+* Elevated [[Erythrocyte sedimentation rate|ESR]]
+* Elevated [[C-reactive protein|CRP]]
+* Elevated [[Antibody|immunoglobulin]]
+|
+* Pleural thickening and [[Plaque|plaques]]
+* [[Calcification]]
+* Nodular or reticular opacities
+* Lobar consolidation
+* [[Atelectasis]]
+* Parenchymal bands
+* Enlarged hilar or mediastinal [[Lymph node|lymph nodes]]
+* Granulomata
+|
+* ↑ [[FEV1/FVC ratio|FEV1/FVC]]
+|
+* ↓ [[Oxygen|O2]]
+* ↑ CO2
+* [[Respiratory acidosis]]
+|
+* Mineral dust +
+|
+* History of environmental exposure and imaging
+* Lung [[biopsy]] not required
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |Radiation−induced lung injury<ref name="pmid25854336">{{cite journal |vauthors=Giridhar P, Mallick S, Rath GK, Julka PK |title=Radiation induced lung injury: prediction, assessment and management |journal=Asian Pac. J. Cancer Prev. |volume=16 |issue=7 |pages=2613–7 |date=2015 |pmid=25854336 |doi= |url=}}</ref>
+| align="center" |Subacute/chronic
+| align="center" |Any age
+| align="center" |Both
+| align="center" |−
+| align="center" |−
+| align="center" | +
+| align="center" |−
+| align="center" | +
+| align="center" |Dry
+| align="center" | +
+| align="center" | +
+| align="center" | +
+|
+* [[Rales|Crackles]]
+* [[Pleural friction rub|Pleural rub]]
+* Dullness to [[percussion]]
+| align="center" | +
+| align="center" |−
+|
+* Nonspecific
+|
+* Perivascular haziness to patchy [[Alveolus|alveolar]] filling densities
+* Straight line effect
+* [[Pleural effusion]]
+|
+* ↓ [[Total lung capacity|TLC]]
+* ↓ [[Vital capacity|FVC]]
+* ↓ [[Spirometry|FEV1]]
+* ↓ [[DLCO]]
+|
+* ↓ [[Pulmonary gas pressures|PaO2]]
+* ↓ [[Oxygen|O2]]
+|
+* [[Lymphocytosis]]
+|
+* History of irradiation and clinical presentation
+|-
+! rowspan="3" style="background:#DCDCDC;" align="center" |Pulmonary hemorrhage syndromes
+! style="background:#DCDCDC;" align="center" |[[Goodpasture syndrome]]<ref name="pmid29083697">{{cite journal |vauthors=DeVrieze BW, Hurley JA |title= |journal= |volume= |issue= |pages= |date= |pmid=29083697 |doi= |url=}}</ref>
+| align="center" |Chronic
+| align="center" |All ages
+| align="center" |Male
+| align="center" | +
+| align="center" |±
+| align="center" | −
+| align="center" |−
+| align="center" |±
+| align="center" |Bloody
+| align="center" |±
+| align="center" |−
+| align="center" |−
+|
+* Bilateral coarse [[crepitations]]
+| align="center" |−
+| align="center" |−
+|
+* [[Anti-glomerular basement membrane antibody|Anti-GBM antibody]] +
+* [[Anti-neutrophil cytoplasmic antibody|ANCA]] +
+* [[Anemia]]
+* [[Red blood cell|RBC]] in the urine
+|
+* Pulmonary infiltrates
+|
+* ↑ [[DLCO]]
+|
+* Normal
+|
+* NA
+|
+* Kidney [[biopsy]]
+|-
+! style="background:#DCDCDC;" align="center" |Idiopathic pulmonary hemosiderosis<ref name="KhorashadiWu2015">{{cite journal|last1=Khorashadi|first1=L.|last2=Wu|first2=C.C.|last3=Betancourt|first3=S.L.|last4=Carter|first4=B.W.|title=Idiopathic pulmonary haemosiderosis: spectrum of thoracic imaging findings in the adult patient|journal=Clinical Radiology|volume=70|issue=5|year=2015|pages=459–465|issn=00099260|doi=10.1016/j.crad.2014.11.007}}</ref>
+| align="center" |Acute/subacute/chronic
+| align="center" |Children − 10 years old
+| align="center" |Both
+| align="center" | +
+| align="center" |±
+| align="center" | −
+| align="center" |−
+| align="center" | +
+| align="center" |Bloody
+| align="center" | +
+| align="center" | +
+| align="center" |−
+|
+* [[Rales|Crackles]]
+| align="center" | −
+| align="center" |−
+|
+* [[Iron deficiency anemia]]
+* ↑ Plasma [[bilirubin]]
+* ↑ Urinary excretion of [[urobilinogen]]
+* ↑ [[Reticulocytes]]
+* [[Fecal occult blood]] +
+|
+* Mid to lower zone [[Alveolus|alveolar]] opacities
+* Multiple honeycomb [[Cyst|cysts]]
+|
+* ↓ [[Lung volumes|TLC]]
+* ↓ [[Vital capacity|FVC]]
+* ↓ [[Spirometry|FEV1]]
+* ↑ [[FEV1/FVC ratio|FEV1/FVC]]
+* ↑ [[DLCO]]
+|
+* ↓ [[Oxygen|O2]]
+* ↓ CO2
+|
+* ↑  [[Hemosiderin]]−laden [[Macrophage|macrophages]]
+|
+* Clinical evaluation and investigations
+|-
+! style="background:#DCDCDC;" align="center" |Isolated pulmonary capillaritis<ref name="ThompsonKlecka2016">{{cite journal|last1=Thompson|first1=Gwen|last2=Klecka|first2=Mary|last3=Roden|first3=Anja C.|last4=Specks|first4=Ulrich|last5=Cartin-Ceba|first5=Rodrigo|title=Biopsy-proven pulmonary capillaritis: A retrospective study of aetiologies including an in-depth look at isolated pulmonary capillaritis|journal=Respirology|volume=21|issue=4|year=2016|pages=734–738|issn=13237799|doi=10.1111/resp.12738}}</ref>
+| align="center" |Chronic
+| align="center" |40−60 years old
+| align="center" |Both
+| align="center" | +
+| align="center" |−
+| align="center" |±
+| align="center" | −
+| align="center" | +
+| align="center" |Bloody
+| align="center" | +
+| align="center" | +
+| align="center" | +
+|
+* Decreased [[breath sounds]]
+| align="center" |−
+| align="center" |−
+|
+* [[Anemia]]
+* [[Leukocytosis]]
+|
+* Diffuse [[Alveolus|alveolar]] haemorrhage
+|
+* ↓ [[FEV1/FVC ratio|FEV1/FVC]]
+|
+* ↓ [[Oxygen|O2]]
+|
+* Diffuse [[Alveolus|alveolar]] haemorrhage
+|
+* Diagnosis of exclusion
+|-
+! colspan="2" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
+! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |History
+! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Symptoms
+! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Physical examination
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab findings
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging
+! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Pulmonary function test
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Bronchoscopy and BAL
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Gold standard
+|-
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Duration
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Age
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Gender
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Family history
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Smoking history
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Environmental exposure
+! style="background:#4479BA; color: #FFFFFF;" align="center" |HIV
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cough
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Wheezing
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Chest pain
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Tachypnea
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Auscultation
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cyanosis
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Clubbing
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Spirometry
+! style="background:#4479BA; color: #FFFFFF;" align="center" |ABG
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |[[Sarcoidosis]]<ref name="LiTao2018">{{cite journal|last1=Li|first1=Cheng-Wei|last2=Tao|first2=Ru-Jia|last3=Zou|first3=Dan-Feng|last4=Li|first4=Man-Hui|last5=Xu|first5=Xin|last6=Cao|first6=Wei-Jun|title=Pulmonary sarcoidosis with and without extrapulmonary involvement: a cross-sectional and observational study in China|journal=BMJ Open|volume=8|issue=2|year=2018|pages=e018865|issn=2044-6055|doi=10.1136/bmjopen-2017-018865}}</ref>
+| align="center" |Acute/subacute/chronic
+| align="center" |20−40 years old
+| align="center" |Female
+| align="center" | +
+| align="center" |±
+| align="center" | −
+| align="center" | −
+| align="center" |±
+| align="center" | +
+| align="center" | +
+| align="center" |±
+| align="center" | −
+|
+* [[Rales|Crackles]]
+* [[Wheeze|Wheezing]]
+* Decreased [[breath sounds]]
+| align="center" | +
+| align="center" | −
+|
+* [[Hypercalciuria]]
+* [[Hypercalcemia]]
+* High [[Angiotensin-converting enzyme|ACE]]
+* [[Hypergammaglobulinemia]]
+|
+* [[Hilar lymphadenopathy|Hilar adenopathy]]
+* Reticular opacities
+* [[Pneumothorax]]
+* [[Pleural cavity|Pleural]] thickening
+* [[Chylothorax]]
+* [[Pulmonary hypertension]]
+|
+* ↓ [[Lung volumes|TLC]]
+* ↓ [[Vital capacity|FVC]]
+* ↓ [[Spirometry|FEV1]]
+* ↑ [[FEV1/FVC ratio|FEV1/FVC]]
+* ↓ [[DLCO]]
+|
+* ↓ [[Oxygen|O2]]
+* ↓ CO2
+* [[Respiratory acidosis]]
+|
+* [[Lymphocytosis]]
+* Elevated [[adenosine deaminase]]
+* [[D-dimer]] +
+|
+* Clinical evaluation and investigations
+|-
+! rowspan="2" style="background:#DCDCDC;" align="center" |Granulomatous vasculitides
+! style="background:#DCDCDC;" align="center" |Granulomatosis with polyangiitis (Wegener)<ref name="pmid26684637">{{cite journal |vauthors=Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, De Virgilio A, Zambetti G, de Vincentiis M |title=Clinic manifestations in granulomatosis with polyangiitis |journal=Int J Immunopathol Pharmacol |volume=29 |issue=2 |pages=151–9 |date=June 2016 |pmid=26684637 |pmc=5806708 |doi=10.1177/0394632015617063 |url=}}</ref>
+| align="center" |Chronic
+| align="center" |Elderly
+| align="center" |Both
+| align="center" | +
+| align="center" | −
+| align="center" | −
+| align="center" | −
+| align="center" | +
+| align="center" | +
+| align="center" | +
+| align="center" |±
+| align="center" | −
+|
+* [[Rales|Crackles]]
+| align="center" | −
+| align="center" | −
+|
+* [[Anti-neutrophil cytoplasmic antibody|ANCA]] +
+* [[Anemia]]
+* [[Leukocytosis]]
+* [[Thrombocytosis]]
+* Elevated [[Erythrocyte sedimentation rate|ESR]]
+* Elevated [[C-reactive protein|CRP]]
+* Elevated [[creatinine]]
+* Urine [[protein]] +
+* [[Hematuria]]
+|
+* Cavitate [[Nodule (medicine)|nodules]]
+* Ground−glass opacity
+* [[Consolidation (medicine)|Consolidation]]
+* [[Pleural effusion]]
+* [[Hilar lymphadenopathy|Hilar adenopathy]]
+|
+* ↓ [[FEV1/FVC ratio|FEV1/FVC]]
+|
+* ↓ [[Oxygen|O2]]
+|
+* Alveolar hemorrhage
+|
+* Lung [[biopsy]]
+|-
+! style="background:#DCDCDC;" align="center" |[[Eosinophilic granulomatosis with polyangiitis]] (Churg Strauss)<ref name="pmid25500434">{{cite journal |vauthors=Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M |title=Churg-Strauss syndrome |journal=Autoimmun Rev |volume=14 |issue=4 |pages=341–8 |date=April 2015 |pmid=25500434 |doi=10.1016/j.autrev.2014.12.004 |url=}}</ref>
+| align="center" |Chronic
+| align="center" |40−50 years old
+| align="center" |Both
+| align="center" | +
+| align="center" | −
+| align="center" | −
+| align="center" | −
+| align="center" | −
+| align="center" | +
+| align="center" | +
+| align="center" | −
+| align="center" | −
+|
+* Scattered [[Wheeze|wheezing]]
+| align="center" | −
+| align="center" | −
+|
+* [[Eosinophilia]]
+* Elevated [[Immunoglobulin E|IgE]] titers
+* [[Anti-neutrophil cytoplasmic antibody|ANCA]] +
+|
+* Areas of parenchymal opacification
+* Mixed interstitial patchy [[Alveolus|alveolar]] opacities
+|
+* ↓ [[Lung volumes|TLC]]
+* ↑ [[Residual volume|RV]]
+|
+* Normal
+|
+* [[Eosinophilia]]
+|
+* Lung [[biopsy]]
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |Bronchocentric granulomatosis<ref name="Myers1989">{{cite journal|last1=Myers|first1=Jeffrey L.|title=Bronchocentric Granulomatosis|journal=Chest|volume=96|issue=1|year=1989|pages=3–4|issn=00123692|doi=10.1378/chest.96.1.3}}</ref>
+| align="center" |Chronic
+| align="center" |30−70 years old
+| align="center" |Both
+| align="center" | −
+| align="center" |−
+| align="center" |−
+| align="center" |−
+| align="center" |±
+| align="center" |±
+| align="center" | +
+| align="center" |±
+| align="center" | −
+|
+* Scattered [[Wheeze|wheezing]]
+| align="center" | −
+| align="center" |−
+|
+* [[Eosinophilia]]
+* Elevated [[Immunoglobulin E|IgE]] titers
+* Elevated circulating IgE antibodies to [[Aspergillus]] species
+|
+* Upper zone single or multiple pulmonary [[Nodule (medicine)|nodules]]
+* [[Consolidation (medicine)|Consolidation]]
+* [[Atelectasis]]
+|
+* ↓ [[FEV1/FVC ratio|FEV1/FVC]]
+|
+* Normal
+|
+* [[Eosinophilia]]
+|
+* Lung [[biopsy]]
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |Pulmonary lymphomatoid granulomatosis<ref name="AnkitaShashi2016">{{cite journal|last1=Ankita|first1=Grover|last2=Shashi|first2=Dhawan|title=Pulmonary Lymphomatoid Granulomatosis- a Case Report with Review of Literature|journal=Indian Journal of Surgical Oncology|volume=7|issue=4|year=2016|pages=484–487|issn=0975-7651|doi=10.1007/s13193-016-0525-1}}</ref>
+| align="center" |Chronic
+| align="center" |30−50 years old
+| align="center" |Male
+| align="center" | −
+| align="center" |−
+| align="center" |−
+| align="center" |−
+| align="center" | +
+| align="center" | +
+| align="center" | +
+| align="center" | +
+| align="center" |−
+|
+* Normal
+| align="center" |−
+| align="center" |−
+|
+* [[Epstein Barr virus|Epstein-Barr virus]] (EBV) serology +
+|
+* Mid to lower zone multiple poorly defined [[Nodule (medicine)|nodules]]
+* Diffuse reticular abnormalities
+|
+* Normal
+|
+* ↓ [[Oxygen|O2]]
+* Chronic [[respiratory alkalosis]]
+|
+* Normal
+|
+* Lung [[biopsy]]
+|-
+! colspan="2" style="background:#DCDCDC;" align="center" |[[Amyloidosis]]<ref name="KhoorColby2017">{{cite journal|last1=Khoor|first1=Andras|last2=Colby|first2=Thomas V.|title=Amyloidosis of the Lung|journal=Archives of Pathology & Laboratory Medicine|volume=141|issue=2|year=2017|pages=247–254|issn=0003-9985|doi=10.5858/arpa.2016-0102-RA}}</ref><ref name="MilaniBasset2017">{{cite journal|last1=Milani|first1=Paolo|last2=Basset|first2=Marco|last3=Russo|first3=Francesca|last4=Foli|first4=Andrea|last5=Palladini|first5=Giovanni|last6=Merlini|first6=Giampaolo|title=The lung in amyloidosis|journal=European Respiratory Review|volume=26|issue=145|year=2017|pages=170046|issn=0905-9180|doi=10.1183/16000617.0046-2017}}</ref>
+| align="center" |Subacute/chronic
+| align="center" |50−70 years old
+| align="center" |Male
+| align="center" | +
+| align="center" | −
+| align="center" | −
+| align="center" | −
+| align="center" | −
+| align="center" | Bloody
+| align="center" | +
+| align="center" | −
+| align="center" | −
+|
+* [[Rales|Crackles]]
+| align="center" | −
+| align="center" | −
+|
+* Congophilia with apple−green birefringence under polarized light
+|
+* Tracheobronchial infiltration
+* Persistent [[Pleural effusion|pleural effusions]]
+* Parenchymal [[Nodule (medicine)|nodules]]
+|
+* ↓ [[FEV1/FVC ratio|FEV1/FVC]]
+|
+* ↓ [[Oxygen|O2]]
+|
+* Normal
+|
+* Lung [[biopsy]]
+|-
+! colspan="2" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
+! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |History
+! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Symptoms
+! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Physical examination
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab findings
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging
+! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Pulmonary function test
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Bronchoscopy and BAL
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Gold standard
+|-
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Duration
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Age
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Gender
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Family history
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Smoking history
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Environmental exposure
+! style="background:#4479BA; color: #FFFFFF;" align="center" |HIV
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cough
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Wheezing
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Chest pain
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Tachypnea
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Auscultation
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cyanosis
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Clubbing
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Spirometry
+! style="background:#4479BA; color: #FFFFFF;" align="center" |ABG
+|}
+</small></small>
+== Laboratory Finidngs ==
+There are multiple laboratory tests that may be helpful to ascertain or rule out the diagnosis of interstitial lung disease.
+{| class="wikitable"
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Condition
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
+! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Investigation
+|-
+| colspan="2" rowspan="7" |All patients with suspicious interstitial lung disease
+| colspan="2" |[[Complete blood count]] and differential
+|-
+| rowspan="3" |[[Liver function tests]]
+|[[Alanine transaminase|Alanine aminotransferase]] (ALT, SGPT)
+|-
+|[[Aspartate transaminase|Aspartate aminotransferase]] (AST, SGOT)
+|-
+|[[Alkaline phosphatase]]
+|-
+| rowspan="3" |[[Renal function tests]]
+|[[Urine|Urinalysis]]
+|-
+|[[Blood urea nitrogen]] (BUN)
+|-
+|[[Creatinine]] (Cr)
+|-
+| rowspan="7" |Suspicious of systemic [[rheumatic disease]]
+|[[Rheumatoid arthritis|RA]]
+|[[Serology]]
+|[[Anti-citrullinated protein antibody|Anti−cyclic citrullinated peptide]] (Anti-CCP)
+|-
+|[[SLE]]
+|[[Serology]]
+|[[Anti-dsDNA antibody|Anti−double stranded DNA antibodies]] (Anti-dsDNA antibody)
+|-
+|[[Dermatomyositis|Amyopathic dermatomyositis]]
+|[[Serology]]
+|Anti-melanoma differentiation-associated gene 5 (MDA-5)
+|-
+| rowspan="4" |Nonspecific
+|[[Serology]]
+|[[Anti-nuclear antibody|Antinuclear antibody]] (ANA)
+|-
+|[[Serology]]
+|[[Rheumatoid factor]] (RF)
+|-
+|[[Serology]]
+|[[Anti-neutrophil cytoplasmic antibody]] (ANCA)
+|-
+|[[Enzyme]]
+|[[Creatine kinase]] (CK), [[aldolase]]
+|-
+| rowspan="3" |Mechanic hands
+| rowspan="3" |[[Myositis]]
+| rowspan="3" |Myositis−associated antibodies
+|Anti-tRNA synthetases Jo-1
+|-
+|Anti-tRNA synthetases PL-7
+|-
+|Anti-tRNA synthetases PL-12
+|-
+| rowspan="4" |[[Sicca syndrome|Sicca]] features or positive anti−extractable nuclear antigen (ENA)
+| rowspan="2" |[[Sjögren's syndrome|Sjögren’s syndrome]]
+|[[Serology]]
+|Anti-RO (SS−A)
+|-
+|[[Serology]]
+|Anti-La (SS−B)
+|-
+|[[Mixed connective tissue disease]]
+|[[Serology]]
+|Anti-ribonucleoprotein (RNP)
+|-
+|IgG4-related disease
+|[[Serology]]
+|Serum IgG4
+|-
+| rowspan="2" |Severe GERD or sclerodactyly
+|Limited systemic [[scleroderma]]
+|[[Serology]]
+|Anti-centromere
+|-
+|Systemic [[scleroderma]]
+|[[Serology]]
+|Anti-topoisomerase I (anti-Scl-70)
+|-
+| rowspan="2" |Dyspnea
+|Heart failure
+| rowspan="2" |[[Enzyme]]
+|[[Brain natriuretic peptide]] (BNP)
+|-
+|[[Pulmonary hypertension]]
+|N-terminal proBNP (NT-proBNP)
+|-
+| rowspan="4" |[[Anemia]] and/or [[hemoptysis]]
+|[[Coagulopathy|Coagulopathies]]
+| colspan="2" |[[Coagulation|Coagulation studies]]
+|-
+|[[Goodpasture syndrome]]
+|[[Serology]]
+|Anti−glomerular basement membrane (GBM) antibodies
+|-
+|[[Antiphospholipid syndrome]]
+|[[Serology]]
+|[[Antiphospholipid antibodies]]
+|-
+|Idiopathic pulmonary hemosiderosis
+|[[Serology]]
+|Serum IgA endomysial or tissue transglutaminase antibodies
+|-
+|Mediastinal lymphadenopathy
+|[[Multiple myeloma]]
+| colspan="2" |Serum protein electrophoresis
+|-
+|Beryllium exposure
+|[[Berylliosis]]
+| colspan="2" |Peripheral blood beryllium lymphocyte proliferation test
+|-
+| rowspan="2" |Risk factors for HIV
+| rowspan="2" |[[HIV]]
+| colspan="2" |[[ELISA]]
+|-
+| colspan="2" |Western blot test
+|}
+== Occupational Lung Disease ==
+* [[Occupational lung disease|Occupational lung diseases]] are caused by the accumulation of different dust particles in the [[Alveolus|alveolar]] space.<ref name="pmid10931786">{{cite journal| author=Castranova V, Vallyathan V| title=Silicosis and coal workers' pneumoconiosis. | journal=Environ Health Perspect | year= 2000 | volume= 108 Suppl 4 | issue=  | pages= 675-84 | pmid=10931786 | doi= | pmc=PMC1637684 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10931786  }} </ref>
+* As the particles accumulate, the elimination mechanisms of the body begin to fail, resulting in activation of [[Chemotaxis|chemotactic]] factors that exacerbate the [[Inflammation|inflammatory]] response, and subsequently lead to [[fibrosis]].
+* The most common particles that cause [[pneumoconiosis]] are [[asbestos]], [[silica]], coal, magnesium silicate, aluminum silicate, [[bauxite]], [[cobalt]], [[beryllium]] and [[iron]].
+'''For more information about occupational lung disease, [[Occupational lung disease|click here]].'''
+== Drug-induced lung injury==
+More than 600 medications have [[Pulmonary toxicity|pulmonary toxicity]] and may cause [[lung]] injury.<ref name="CamusBonniaud2004">{{cite journal|last1=Camus|first1=Philippe|last2=Bonniaud|first2=Philippe|last3=Fanton|first3=Annlyse|last4=Camus|first4=Clio|last5=Baudaun|first5=Nicolas|last6=Foucher|first6=Pascal|title=Drug-induced and iatrogenic infiltrative lung disease|journal=Clinics in Chest Medicine|volume=25|issue=3|year=2004|pages=479–519|issn=02725231|doi=10.1016/j.ccm.2004.05.006}}</ref>
+* [[Lung]] injury following medication intake might vary from interstitial lung disease to [[hypersensitivity pneumonitis]], [[pleural effusion]] or [[pulmonary edema]].<ref name="pmid8484641">{{cite journal |vauthors=Todd NW, Peters WP, Ost AH, Roggli VL, Piantadosi CA |title=Pulmonary drug toxicity in patients with primary breast cancer treated with high-dose combination chemotherapy and autologous bone marrow transplantation |journal=Am. Rev. Respir. Dis. |volume=147 |issue=5 |pages=1264–70 |date=May 1993 |pmid=8484641 |doi=10.1164/ajrccm/147.5.1264 |url=}}</ref><ref name="pmid15062605">{{cite journal |vauthors=Schwarz MI, Fontenot AP |title=Drug-induced diffuse alveolar hemorrhage syndromes and vasculitis |journal=Clin. Chest Med. |volume=25 |issue=1 |pages=133–40 |date=March 2004 |pmid=15062605 |doi=10.1016/S0272-5231(03)00139-4 |url=}}</ref>
+* The presentation of [[lung]] injury might be acute, subacute or chronic and it might occur weeks to years even after discontinuing the drug.<ref name="pmid9493644">{{cite journal |vauthors=De Vuyst P, Pfitzenmeyer P, Camus P |title=Asbestos, ergot drugs and the pleura |journal=Eur. Respir. J. |volume=10 |issue=12 |pages=2695–8 |date=December 1997 |pmid=9493644 |doi= |url=}}</ref><ref name="pmid20592596">{{cite journal |vauthors=Wijnen PA, Bekers O, Drent M |title=Relationship between drug-induced interstitial lung diseases and cytochrome P450 polymorphisms |journal=Curr Opin Pulm Med |volume=16 |issue=5 |pages=496–502 |date=September 2010 |pmid=20592596 |doi=10.1097/MCP.0b013e32833c06f1 |url=}}</ref>
+* Diagnosis of drug-induced lung injury is by the exclusion of other diseases. Detailed history and paraclinical [[Lung|pulmonary]] investigations are required to exclude other causes of interstitial lung disease. [[Lung|Pulmonary]] investigations are as follow:<ref name="Matsuno2012">{{cite journal|last1=Matsuno|first1=Osamu|title=Drug-induced interstitial lung disease: mechanisms and best diagnostic approaches|journal=Respiratory Research|volume=13|issue=1|year=2012|pages=39|issn=1465-9921|doi=10.1186/1465-9921-13-39}}</ref>
+** [[Restrictive lung disease|Restrictive pattern]] on [[Spirometry|pulmonary function tests]] ([[Spirometry|PFTs]])
+** [[FEV1/FVC ratio]] normal or increased
+** Reduced [[DLCO]]
+** [[Bronchoalveolar lavage]] may exclude [[Infection|infectious]] disease
+** [[Bronchoscopy]] with transbronchial [[biopsy]] may be indicated to exclude other [[Lung|pulmonary]] diseases
+** Invasive lung [[biopsy]] rarely required
+* Management of drug-induced lung injury includes:<ref name="pmid205227932">{{cite journal |vauthors=Gingo MR, George MP, Kessinger CJ, Lucht L, Rissler B, Weinman R, Slivka WA, McMahon DK, Wenzel SE, Sciurba FC, Morris A |title=Pulmonary function abnormalities in HIV-infected patients during the current antiretroviral therapy era |journal=Am. J. Respir. Crit. Care Med. |volume=182 |issue=6 |pages=790–6 |date=September 2010 |pmid=20522793 |pmc=2949404 |doi=10.1164/rccm.200912-1858OC |url=}}</ref>
+** Immediate drug discontinuation
+** Supportive therapy
+** Control of other underlying chronic [[Pulmonology|pulmonary disease]]
+** Treatment of [[Respiratory tract infection|respiratory infections]]
+** [[Smoking cessation]]
+** [[Glucocorticoid]] therapy<ref name="pmid7842781">{{cite journal |vauthors=Kalaycioglu M, Kavuru M, Tuason L, Bolwell B |title=Empiric prednisone therapy for pulmonary toxic reaction after high-dose chemotherapy containing carmustine (BCNU) |journal=Chest |volume=107 |issue=2 |pages=482–7 |date=February 1995 |pmid=7842781 |doi= |url=}}</ref>
+* List of medications that might cause interstitial lung disease is as follow:<ref name="Schwaiblmair2012">{{cite journal|last1=Schwaiblmair|first1=Martin|title=Drug Induced Interstitial Lung Disease|journal=The Open Respiratory Medicine Journal|volume=6|issue=1|year=2012|pages=63–74|issn=18743064|doi=10.2174/1874306401206010063}}</ref><ref name="pmid25546981">{{cite journal |vauthors=Vasić NR, Milenković BA, Pešut DP, Stević RS, Jovanović DM |title=Drug induced lung disease--amiodarone in focus |journal=Med. Pregl. |volume=67 |issue=9-10 |pages=334–7 |date=2014 |pmid=25546981 |doi= |url=}}</ref>
+{| class="wikitable"
+! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |Drug−induced lung injury
+|-
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Antimicrobial Agents
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Anti−Inflammatory Agents
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Biological Agents
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Cardiovascular Agents
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Immunomodulator agents
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Antineoplastic agents
+! style="background:#4479BA; color: #FFFFFF;" align="center" |Miscellaneous
+|- valign="top"
+|
+* [[Amphotericin B]]
+* [[Doripenem]]
+* [[Ethambutol]]
+* [[Isoniazid]]
+* [[Minocycline]]
+* [[Nitrofurantoin]]
+* [[Sulfasalazine]]
+|
+* [[Abatacept]]
+* [[Aspirin]]
+* [[Azathioprine]]
+* [[Cyclophosphamide]]
+* [[Gold]]
+* [[Anakinra]]
+* [[Leflunomide]]
+* [[Methotrexate]]
+* [[Non-steroidal anti-inflammatory drug|Nonsteroidal anti-inflammatory drugs]]
+* [[Penicillamine]]
+* [[Sulfasalazine]]
+* [[Thalidomide]]
+|
+* [[Adalimumab]]
+* [[Alemtuzumab]]
+* [[Interferon type I|Alpha interferon]]
+* [[Bevacizumab]]
+* [[Cetuximab]]
+* [[Etanercept]]
+* [[Infliximab]]
+* [[Panitumumab]]
+* [[Rituximab]]
+* [[Tocilizumab]]
+* [[Trastuzumab]]
+|
+* [[ACE inhibitor|ACE inhibitors]]
+* [[Amiodarone]]<ref name="pmid19399307">{{cite journal |vauthors=Wolkove N, Baltzan M |title=Amiodarone pulmonary toxicity |journal=Can. Respir. J. |volume=16 |issue=2 |pages=43–8 |date=2009 |pmid=19399307 |pmc=2687560 |doi= |url=}}</ref>
+* [[Anticoagulant|Anticoagulants]]
+* [[Beta blockers]]
+* [[Flecainide]]
+* [[Hydrochlorothiazide]]
+* [[Procainamide]]
+* [[Statins]]<ref name="FernándezKaras2008">{{cite journal|last1=Fernández|first1=Antonio B.|last2=Karas|first2=Richard H.|last3=Alsheikh-Ali|first3=Alawi A.|last4=Thompson|first4=Paul D.|title=Statins and Interstitial Lung Disease|journal=Chest|volume=134|issue=4|year=2008|pages=824–830|issn=00123692|doi=10.1378/chest.08-0943}}</ref>
+* [[Tocainide]]
+|
+* [[Sirolimus]]
+* [[Riluzole]]
+* [[Trametinib]]
+* [[vandetanib]]
+|
+<div style="-moz-column-count:2; column-count:2;">
+* [[Azathioprine]]
+* [[Bleomycin]]
+* [[Bortezomib]]
+* [[Busulfan]]
+* [[Carmustine]] (BCNU)<ref name="pmid2370889">{{cite journal |vauthors=O'Driscoll BR, Hasleton PS, Taylor PM, Poulter LW, Gattameneni HR, Woodcock AA |title=Active lung fibrosis up to 17 years after chemotherapy with carmustine (BCNU) in childhood |journal=N. Engl. J. Med. |volume=323 |issue=6 |pages=378–82 |date=August 1990 |pmid=2370889 |doi=10.1056/NEJM199008093230604 |url=}}</ref>
+* [[Chlorambucil]]
+* [[Colony-stimulating factor|Colony-stimulating factors]]
+* [[Crizotinib]]
+* [[Cyclophosphamide]]<ref name="pmid84846412">{{cite journal |vauthors=Todd NW, Peters WP, Ost AH, Roggli VL, Piantadosi CA |title=Pulmonary drug toxicity in patients with primary breast cancer treated with high-dose combination chemotherapy and autologous bone marrow transplantation |journal=Am. Rev. Respir. Dis. |volume=147 |issue=5 |pages=1264–70 |date=May 1993 |pmid=8484641 |doi=10.1164/ajrccm/147.5.1264 |url=}}</ref>
+* [[Cytarabine]]
+* [[Deferoxamine]]
+* [[Docetaxel]]
+* [[Doxorubicin hydrochloride|Doxorubicin]]
+* [[Eribulin]]
+* [[Erlotinib]]
+* [[Etoposide]] (VP−16)
+* [[Fludarabine]]
+* [[Flutamide]]
+* [[Gefitinib]]
+* [[Gemcitabine]]<ref name="pmid23404815">{{cite journal |vauthors=Tamura M, Saraya T, Fujiwara M, Hiraoka S, Yokoyama T, Yano K, Ishii H, Furuse J, Goya T, Takizawa H, Goto H |title=High-resolution computed tomography findings for patients with drug-induced pulmonary toxicity, with special reference to hypersensitivity pneumonitis-like patterns in gemcitabine-induced cases |journal=Oncologist |volume=18 |issue=4 |pages=454–9 |date=2013 |pmid=23404815 |pmc=3639533 |doi=10.1634/theoncologist.2012-0248 |url=}}</ref>
+* [[Hydroxyurea]]
+* [[Imatinib]]
+* [[Lomustine]] (CCNU)
+* [[Melphalan]]
+* [[Mitomycin (patient information)|Mitomycin-C]]
+* [[Nilutamide]]
+* [[Nitrosourea]]
+* [[Olsalazine]]
+* [[Paclitaxel]]
+* [[Procarbazine]]
+* [[Semustine]] (Methyl−CCNU)
+* [[Sorafenib]]
+* [[Vinblastine]]
+|
+* [[BCG vaccine|Bacille Calmette-Guerin]] (BCG)
+* [[Bromocriptine]]
+* [[Carbamazepine]]
+* [[Cabergoline]]
+* [[Cocaine]]<ref name="pmid22934773">{{cite journal |vauthors=Drent M, Wijnen P, Bast A |title=Interstitial lung damage due to cocaine abuse: pathogenesis, pharmacogenomics and therapy |journal=Curr. Med. Chem. |volume=19 |issue=33 |pages=5607–11 |date=2012 |pmid=22934773 |doi= |url=}}</ref>
+* [[Methysergide]]
+* [[Tryptophan|L-tryptophan]]
+* [[Penicillamine]]
+* [[Phenytoin]]
+* [[Talcosis|Talc]]
+|}
+== Radiation-induced Lung Injury ==
+[[Radiation therapy|Radiation]] has been considered as one of the causes of [[lung]] injury. About 5 to 15% of patients receiving [[radiation therapy]] may present with [[Lung|pulmonary]] symptoms.<ref name="MalaviyaGow2015">{{cite journal|last1=Malaviya|first1=Rama|last2=Gow|first2=Andrew J.|last3=Francis|first3=Mary|last4=Abramova|first4=Elena V.|last5=Laskin|first5=Jeffrey D.|last6=Laskin|first6=Debra L.|title=Radiation-Induced Lung Injury and Inflammation in Mice: Role of Inducible Nitric Oxide Synthase and Surfactant Protein D|journal=Toxicological Sciences|volume=144|issue=1|year=2015|pages=27–38|issn=1096-0929|doi=10.1093/toxsci/kfu255}}</ref>
+* [[Lung|Pulmonary]] injury following [[irradiation]] is directly related to duration and dose of [[Radiation therapy|radiation]].<ref name="pmid16015535">{{cite journal |vauthors=Kong FM, Ten Haken R, Eisbruch A, Lawrence TS |title=Non-small cell lung cancer therapy-related pulmonary toxicity: an update on radiation pneumonitis and fibrosis |journal=Semin. Oncol. |volume=32 |issue=2 Suppl 3 |pages=S42–54 |date=April 2005 |pmid=16015535 |doi= |url=}}</ref>
+* The main pathogenesis of radiation-induced lung injury is the damage to the type I [[Pneumocyte|pneumocytes]] which triggers the initiation of reactions. It leads to secretion of [[Growth factor|growth factors]] and [[Protease|proteases]] which increases degradation of [[extracellular matrix]]. Also, radiation causes damage to [[Epithelium|epithelial cells]] which leads to loss of barrier function. All of these changes cause a cycle of [[inflammation]] and [[fibrosis]].<ref name="pmid25854336">{{cite journal |vauthors=Giridhar P, Mallick S, Rath GK, Julka PK |title=Radiation induced lung injury: prediction, assessment and management |journal=Asian Pac. J. Cancer Prev. |volume=16 |issue=7 |pages=2613–7 |date=2015 |pmid=25854336 |doi= |url=}}</ref>
+* There are two types of reaction in [[Lung|pulmonary]] tissues to [[Radiation therapy|radiation]] which include:<ref name="pmid17126203">{{cite journal |vauthors=Tsoutsou PG, Koukourakis MI |title=Radiation pneumonitis and fibrosis: mechanisms underlying its pathogenesis and implications for future research |journal=Int. J. Radiat. Oncol. Biol. Phys. |volume=66 |issue=5 |pages=1281–93 |date=December 2006 |pmid=17126203 |doi=10.1016/j.ijrobp.2006.08.058 |url=}}</ref>
+** Early [[Lung|pulmonary]] reaction:
+*** It usually occurs in 4 to 12 weeks following [[irradiation]].
+*** Early reaction mostly presents as radiation [[pneumonitis]].
+*** Treatment of radiation [[pneumonitis]] include [[Steroid|steroids]], [[ACE inhibitor|ACE inhibitors]] and [[pentoxifylline]].
+** Late [[Lung|pulmonary]] reaction:
+*** It usually occurs 6 to 12 months following [[irradiation]].
+*** Late response usually induces lung [[fibrosis]].
+*** Management of lung [[fibrosis]] consists of supportive therapy, [[airway]] secretions mobilization, anti-inflammatory therapy, and management of acute exacerbations.
+* [[Computed tomography|CT]] might be used for the diagnosis of radiation [[pneumonitis]]. Ground glass opacities, [[Consolidation (medicine)|consolidation]], [[fibrosis]], [[atelectasis]], pulmonary volume loss, or [[Pleural cavity|pleural]] thickening might be seen on [[Computed tomography|CT scan]].
+== Smoking related Interstitial Lung Disease ==
+[[Smoking|Cigarette smoking]] may cause various adverse effects on [[Lung|pulmonary]] tissue.<ref>{{cite journal |last=Nagai |first=Sonoko |coauthors=Yuma Hoshino, Michio Hayashi, Isao Ito |title=Smoking-related interstitial lung diseases |url=http://www.co-pulmonarymedicine.com/pt/re/copulmonary/abstract.00063198-200009000-00005.htm |journal=Current Opinion in Pulmonary Medicine |volume=6 |issue=5 |pages=415-9 |year=2000 |pmid=10958232}}</ref><ref>{{cite journal |last=Baumgartner |first=KB |coauthors=Samet JM, Stidley CA, Colby TV, Waldron JA |title=Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis |url= http://ajrccm.atsjournals.org/cgi/content/short/155/1/242 |journal=American Journal of Respiratory and Critical Care Medicine |volume=155 |number=1 |pages=242-248 |year=1997 |pmid=9001319}}</ref><ref name="pmid29222007">{{cite journal |vauthors=Kumar A, Cherian SV, Vassallo R, Yi ES, Ryu JH |title=Current Concepts in Pathogenesis, Diagnosis, and Management of Smoking-Related Interstitial Lung Diseases |journal=Chest |volume= |issue= |pages= |date=December 2017 |pmid=29222007 |doi=10.1016/j.chest.2017.11.023 |url=}}</ref>
+* [[Smoking|Cigarette smoke]] might injure [[Alveolus|alveolar]] [[Epithelium|epithelial cells]] leading to in diffuse [[Infiltration (medical)|infiltration]] and subsequently [[Parenchyma|parenchymal]] [[fibrosis]].
+* Alveolar [[Epithelium|epithelial cells]] are the primary [[Cell (biology)|cells]] involved in the pathogenesis of [[emphysema]] as well as lung [[fibrosis]] following [[smoking]].
+* [[Smoking|Cigarette smoking]] may predispose alveolar [[Epithelium|epithelial cells]] to cellular [[senescence]] and [[apoptosis]] that leads to [[emphysema]] or lung [[fibrosis]].
+* A well-known association exists between [[tobacco smoking]] and some types of interstitial lung disease such as:
+** Desquamative interstitial pneumonia
+** Respiratory bronchiolitis-associated [[interstitial lung disease]]
+** Pulmonary [[Langerhans cell histiocytosis|Langerhans cell granulomatosis]]
+** Acute [[eosinophilic pneumonia]]
+== Idiopathic Interstitial Pneumonia ==
+The idiopathic interstitial pneumonias (IIP) are a broad range of interstitial lung diseases of unknown etiology.<ref name="Selman">{{cite journal |last=Selman |first=Moisés |coauthors=Talmadge E. King, Jr.; and Annie Pardo |title=Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy |journal=Annals of Internal Medicine |year=2001 |volume=134 |number=2 |pages=136-51|url=http://www.annals.org/cgi/content/abstract/134/2/136}}</ref><ref>{{cite journal |last=King, Jr. |first=Talmadge E. |title=Centennial review: clinical advances in the diagnosis and therapy of the interstitial lung diseases|url=http://ajrccm.atsjournals.org/cgi/content/full/172/3/268 |journal=American Journal of Respiratory and Critical Care Medicine |year=2005 |volume=172 |number=3 |pages=268-79}}</ref><ref name="Flaherty-2001">{{Cite journal | last1 = Flaherty | first1 = KR. | last2 = Travis | first2 = WD. | last3 = Colby | first3 = TV. | last4 = Toews | first4 = GB. | last5 = Kazerooni | first5 = EA. | last6 = Gross | first6 = BH. | last7 = Jain | first7 = A. | last8 = Strawderman | first8 = RL. | last9 = Flint | first9 = A. | title = Histopathologic variability in usual and nonspecific interstitial pneumonias. | journal = Am J Respir Crit Care Med | volume = 164 | issue = 9 | pages = 1722-7 | month = Nov | year = 2001 | doi = 10.1164/ajrccm.164.9.2103074 | PMID = 11719316 }}</ref><ref name="Cottin-1998">{{Cite journal | last1 = Cottin | first1 = V. | last2 = Donsbeck | first2 = AV. | last3 = Revel | first3 = D. | last4 = Loire | first4 = R. | last5 = Cordier | first5 = JF. | title = Nonspecific interstitial pneumonia. Individualization of a clinicopathologic entity in a series of 12 patients. | journal = Am J Respir Crit Care Med | volume = 158 | issue = 4 | pages = 1286-93 | month = Oct | year = 1998 | doi = 10.1164/ajrccm.158.4.9802119 | PMID = 9769293 }}</ref><ref name="Park-1996">{{Cite journal | last1 = Park | first1 = CS. | last2 = Jeon | first2 = JW. | last3 = Park | first3 = SW. | last4 = Lim | first4 = GI. | last5 = Jeong | first5 = SH. | last6 = Uh | first6 = ST. | last7 = Park | first7 = JS. | last8 = Choi | first8 = DL. | last9 = Jin | first9 = SY. | title = Nonspecific interstitial pneumonia/fibrosis: clinical manifestations, histologic and radiologic features. | journal = Korean J Intern Med | volume = 11 | issue = 2 | pages = 122-32 | month = Jun| year = 1996 | doi = | PMID = 8854648 }}</ref><ref name="Shimizu-2002">{{Cite journal | last1 = Shimizu | first1 = S. | last2 = Yoshinouchi | first2 = T. | last3 = Ohtsuki | first3 = Y. | last4 = Fujita | first4 = J. |last5 = Sugiura | first5 = Y. | last6 = Banno | first6 = S. | last7 = Yamadori | first7 = I. | last8 = Eimoto | first8 = T. | last9 = Ueda | first9 = R. | title = The appearance of S-100 protein-positive dendritic cells and the distribution of lymphocyte subsets in idiopathic nonspecific interstitial pneumonia. | journal = Respir Med | volume = 96 | issue = 10 | pages = 770-6 | month = Oct | year = 2002 | doi = | PMID = 12412975 }}</ref>
+*Pathogenesis of idiopathic interstitial pneumonia is [[Fibroblast|fibroblasts]] proliferation and [[collagen]] deposition leading to [[inflammation]] and [[fibrosis]].
+*Patients with idiopathic interstitial pneumonia generally manifest as a gradual [[dyspnea]] and dry [[cough]].
+*On chest imaging it is characterized by bilateral abnormal opacities of various types.
+*The most common type of idiopathic interstitial pneumonia is chronic [[idiopathic pulmonary fibrosis]]. However, [[Hamman-Rich syndrome|acute interstitial pneumonia]] has the worst prognosis.
+*The exact [[diagnosis]] can only be reached with a multidisciplinary approach after excluding known causes.
+*The new classification of idiopathic interstitial pneumonias is as follow:
+** Major idiopathic interstitial pneumonias
+*** [[Idiopathic pulmonary fibrosis]]
+*** Idiopathic nonspecific interstitial pneumonia
+*** [[Respiratory bronchiolitis-interstitial lung disease]]
+*** [[Desquamative interstitial pneumonia]]
+*** [[Cryptogenic organizing pneumonia]]
+*** [[Hamman-Rich syndrome|Acute interstitial pneumonia]]
+** Rare idiopathic interstitial pneumonias
+*** Idiopathic [[lymphoid interstitial pneumonia]]
+*** Idiopathic pleuroparenchymal fibroelastosis
+** Unclassifiable idiopathic interstitial pneumonias
+'''For more information about Idiopathic interstitial pneumonia, [[Idiopathic interstitial pneumonia|click here]].'''
+'''For more information about idiopathic pulmonary fibrosis, [[Idiopathic pulmonary fibrosis|click here]].'''
+'''For more information about Cryptogenic organizing pneumonia, [[Cryptogenic organizing pneumonia|click here]].'''
+== Pulmonary Alveolar Proteinosis ==
+[[Pulmonary alveolar proteinosis]] (PAP) is a rare lung disease that is characterized by the intra-alveolar accumulation of [[Pulmonary surfactant|surfactant]] phospholipid and [[apoproteins]].<ref name="pmid25864717">{{cite journal |vauthors=Papiris SA, Tsirigotis P, Kolilekas L, Papadaki G, Papaioannou AI, Triantafillidou C, Papaporfyriou A, Karakatsani A, Kagouridis K, Griese M, Manali ED |title=Pulmonary alveolar proteinosis: time to shift? |journal=Expert Rev Respir Med |volume=9 |issue=3 |pages=337–49 |date=June 2015 |pmid=25864717 |doi=10.1586/17476348.2015.1035259 |url=}}</ref><ref name="pmid27514590">{{cite journal |vauthors=Suzuki T, Trapnell BC |title=Pulmonary Alveolar Proteinosis Syndrome |journal=Clin. Chest Med. |volume=37 |issue=3 |pages=431–40 |date=September 2016 |pmid=27514590 |doi=10.1016/j.ccm.2016.04.006 |url=}}</ref>
+* The exact etiology of [[pulmonary alveolar proteinosis]] is unknown.
+* The primary pathogenesis of [[pulmonary alveolar proteinosis]] involves reduction in [[granulocyte-macrophage colony-stimulating factor]] ([[Granulocyte macrophage colony stimulating factor|GM-CSF]]) levels or function and/or impaired alveolar [[macrophage]] function.
+*  Terminal [[Bronchiole|bronchioles]] and [[Pulmonary alveolus|alveoli]] are filled with a lipoproteinaceous material that will be [[Periodic acid-Schiff stain|periodic acid-Schiff]] ([[Periodic acid-Schiff stain|PAS]]) stain positive.
+* If left untreated, patients with [[pulmonary alveolar proteinosis]] may progress to develop pulmonary [[fibrosis]] or [[Right heart failure|cor pulmonale]].
+'''For more information about pulmonary alveolar proteinosis, [[Pulmonary alveolar proteinosis|click here]].'''
+== Lymphocytic Infiltrative Disorders ==
+Lymphocytic infiltrative disorders might cause interstitial lung disease in mostly [[Human Immunodeficiency Virus (HIV)|HIV]] positive children.<ref name="pmid27514593">{{cite journal |vauthors=Panchabhai TS, Farver C, Highland KB |title=Lymphocytic Interstitial Pneumonia |journal=Clin. Chest Med. |volume=37 |issue=3 |pages=463–74 |date=September 2016 |pmid=27514593 |doi=10.1016/j.ccm.2016.04.009 |url=}}</ref><ref name="pmid9363179">{{cite journal |vauthors=Fishback N, Koss M |title=Update on lymphoid interstitial pneumonitis |journal=Curr Opin Pulm Med |volume=2 |issue=5 |pages=429–33 |date=September 1996 |pmid=9363179 |doi= |url=}}</ref>
+* The etiology of lymphocytic infiltrative disorders is unknown. However, there is an evidence of infectious cause such as [[Epstein Barr virus|EBV]] in [[Human Immunodeficiency Virus (HIV)|HIV]] positive patients.
+* The two main manifestations of lymphocytic infiltrative disorders include:
+** [[Lymphocytic interstitial pneumonia|Lymphocytic interstitial pneumonitis]]
+** Pulmonary lymphomatoid granulomatosis
+'''For more information about lymphocytic interstitial pneumonitis, [[Lymphocytic interstitial pneumonitis|click here]].'''
+== Pulmonary Lymphangioleiomyomatosis ==
+Lymphangiomyocytosis (LAM) is defined as a multifocal neoplasm with differentiation of the perivascular epithelioid cell that involves multiple organs.<ref name="pmid29487252">{{cite journal |vauthors=Verma AK, Joshi A, Mishra AR, Kant S, Singh A |title=Pulmonary lymphangioleiomyomatosis presenting as spontaneous pneumothorax treated with sirolimus - A case report |journal=Lung India |volume=35 |issue=2 |pages=154–156 |date=2018 |pmid=29487252 |doi=10.4103/lungindia.lungindia_60_17 |url=}}</ref>
+* Lymphangiomyomatosis is the result of disorderly [[smooth muscle]] proliferation throughout the [[Bronchiole|bronchioles]], [[Alveolus|alveolar]] septa, perivascular spaces, and [[Lymphatic system|lymphatics]], resulting in the obstruction of small airways (leading to pulmonary [[cyst]] formation and [[pneumothorax]]) and [[Lymphatic system|lymphatics]] (leading to [[Chyle|chylous]] [[pleural effusion]]).
+* [[LAM]] occurs in a sporadic form, which only affects females, who are usually of childbearing age.
+'''For more information about pulmonary lymphangioleiomyomatosis, [[Lymphangiomyomatosis|click here]].'''
+== Pulmonary Hemorrhage Syndromes ==
+Pulmonary hemorrhage syndromes might cause interstitial lung disease.
+* Several pulmonary hemorrhage syndromes that affect the lung [[parenchyma]] and eventually lead to pulmonary [[fibrosis]]. Some of these are as follows:
+** [[Goodpasture syndrome]]
+** Idiopathic pulmonary hemosiderosis
+** Isolated pulmonary capillaritis
+== Interstitial Lung Disease Associated with Systemic Diseases ==
+=== Interstitial lung disease associated with connective tissue diseases ===
+* [[Systemic lupus erythematosus]]
+* [[Rheumatoid arthritis]]
+* [[Ankylosing spondylitis]]
+* [[Systemic sclerosis]]
+* [[Sjögren syndrome]]
+* [[Polymyositis]]/[[dermatomyositis]]
+* [[Granulomatosis with polyangiitis]] (Wegener's)
+* [[Eosinophilic granulomatosis with polyangiitis]] (Churg Strauss)
+=== Interstitial lung disease associated with inherited diseases ===
+* [[Tuberous sclerosis]]
+* [[Neurofibromatosis]]
+* [[Niemann−Pick disease]]
+* [[Gaucher disease]]
+* [[Hermansky−Pudlak syndrome]]
+=== Interstitial lung disease associated with gastrointestinal or liver diseases ===
+* [[Crohn disease]]
+* [[Primary biliary cirrhosis]]
+* Chronic active [[hepatitis]]
+* [[Ulcerative colitis]]
+=== Interstitial lung disease associated with graft−versus−host disease ===
+* [[Bone marrow transplantation]]
+* Solid [[organ transplantation]]
-[[de:Interstitielle Lungenerkrankung]]
+== Granulomatous Lung Response ==
-[[es:Enfermedad pulmonar intersticial]]
+* [[Hypersensitivity pneumonitis]]
-[[ja:間質性肺炎]]
+* [[Sarcoidosis]]
-[[fi:Keuhkoparenkyymisairaudet]]
+* Granulomatous vasculitides
+** [[Granulomatosis with polyangiitis]] (Wegener)
+** [[Eosinophilic granulomatosis with polyangiitis]] (Churg Strauss)
+* Bronchocentric granulomatosis
+'''For more information about hypersensitivity pneumonitis, [[Hypersensitivity pneumonitis|click here]].'''
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+==References==
-{{WikiDoc Sources}}
+{{reflist|2}}