Interstitial lung disease

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Interstitial Lung Disease
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Patient Information
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

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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

Overview

Interstitial lung disease , refers to a group of lung diseases (including idiopathic pulmonary fibrosis), affecting the alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues. The term ILD is used to distinguish these diseases from obstructive airways diseases. Most types of ILD involve fibrosis, but this is not essential; indeed fibrosis is often a later feature. Hence the term pulmonary fibrosis has fallen out of favor.

Causes

ILD may be classified according to the cause: -

1. Inhaled substances
   • Inorganic
     • Silica
     • Asbestos
     • Beryllium
   • Organic
     • Hypersensitivity pneumonitis
2. Drug induced
   • Antibiotics
   • Chemotherapeutic drugs
   • Antiarrhythmic agents
3. Connective tissue disease
   • Systemic sclerosis
   • Dermatomyositis
   • Systemic lupus erythematosus
   • Rheumatoid arthritis
4. Infection
   • Atypical pneumonia
   • Pneumocystis pneumonia (PCP)
   • Tuberculosis
5. Idiopathic
   • Sarcoidosis
   • Idiopathic pulmonary fibrosis
   • Hamman-Rich syndrome
6. Malignancy
   • Lymphangitic carcinomatosis

Diagnosis

Patients with pneumocystis pneumonia can present with interstitial lung disease, as seen in the reticular markings on this AP chest x-ray

Investigation is tailored towards the symptoms and signs. Most patients have blood testing, chest x-ray, pulmonary function testing, and high resolution CT thorax.

Treatment

ILD is not a single disease, but encompasses many different pathological processes. Hence treatment is different for each disease.

If a specific occupational exposure cause is found, the person should avoid that environment. If a drug cause is suspected, that drug should be discontinued.

Many idiopathic and connective tissue-based causes of ILD are treated with prednisolone. Some patients respond to immunosuppressant treatment. Patients with hypoxemia may be given supplemental oxygen.

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