Idiopathic pulmonary fibrosis overview
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Differentiating Idiopathic pulmonary fibrosis from other Diseases |
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Diagnosis |
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Treatment |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Historical Perspective
Pulmonary fibrosis was first dexcribed by Dr. Von Buhl in 1872. Dr. Rindfleish reported a case of a 40 year old patient who presented with worsening cough and dyspnea and he named this case as "Cirrhosis cystica pulmonum" in 1898.
Classification
Idiopathic pulmonary fibrosis is a disease among a large group of the interstitial lung diseases and particularly a subtype of the idiopathic interstitial pneumonia. The major group Acute Interstitial Pneumonia (AIP), Cryptogenic Organizing Pneumonia (COP), Respiratory Bronchiolitis–Interstitial Lung Disease (RB-ILD), and Desquamative Interstitial Pneumonia (DIP)
Pathophysiology
Pulmonary fibrosis share the pathogenesis process of interstitial lung disease which involve the pulmonary parenchyma. Although the exact pathogenesis is not fully understood, there are many initiating factors which cause the pulmonary tissue injury. The main features of the lung injury includes inflammation, fibrosis, and granulomas development.
Causes
Common causes of pulmonary fibrosis include autoimmunity, rheumatoid arthritis, scleroderma, tuberculosis, SLE, sarocoidosis, and polymyoisitis. Other causes of pulmonary fibrosis include environmental factors as cigarette smoking and dust exposure.
Differentiating Idiopathic Pulmonary Fibrosis from Other Diseases
Idiopathic pulmonary fibrosis must be differentiated from other causes of interstitial lung diseases which may cause fibrosis as well. Other diseases may include acute interstitial pneumonia, hypersensitivity pneumonia, occupational lung diseases, and pulmonary hemorrhage diseases.
Epidemiology and Demographics
Prevalence of idiopathic pulmonary fibrosis ranges from a low of 0.5 per 100.000 persons to a high of 27.9 per 100.000. Incidence of idiopathic pulmonary fibrosis ranges from a low of 3 per 100,000 persons to a high of 9 per 100,000 persons. The prevalence of idiopathic pulmonary fibrosis increases with age. Idiopathic pulmonary fibrosis is more prevalent in men more than women.
Risk Factors
Common risk factors of pulmonary fibrosis include cigarette smoking and genetic mutations which include hTERT, MUC5B, TERT, and RTEL1. Other risk factors include GERD and wood dust.
Screening
There is insufficient evidence to recommend routine screening for idiopathic pulmonary fibrosis.
Natural History, Complications, and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
There are no diagnostic laboratory findings associated with pulmonary fibrosis. However, useful laboratory findings consistent with the diagnosis of pulmonary fibrosis include abnormal arterial blood gases, sputum analysis, and blood picture.
X-ray
X ray imaging does not show specific features for idiopathic pulmonary fibrosis. However, patients with idiopathic pulmonary fibrosis have imaging abnormalities from the beginning of the disease course.
CT scan
MRI
Other Imaging Findings
There are no other imaging findings associated with idiopathic pulmonary fibrosis.
Other Diagnostic Studies
There are no other diagnostic studies associated with idiopathic pulmonary fibrosis.