Pulmonology MKSAPTable 7.1 Clinical Classification of the Interstitial Lung Diseases

Idiopathic Fibrotic Diseases

Acute interstitial pneumonitis

Idiopathic pulmonary fibrosis

Familial idiopathic pulmonary fibrosis

Lymphocytic interstitial pneumonitis

Bronchiolitis obliterans organizing pneumonia (BOOP)

Connective Tissue Diseases

Scleroderma

Rheumatoid arthritis

Polymyositis-dermatomyositis

Lupus erythematosus

Mixed connective tissue disease

Ankylosing spondylitis

Sjögren's syndrome

Primary (Unrelated) Diseases

Sarcoidosis

Eosinophilic granuloma

Lymphangitic carcinomatosis

Alveolar proteinosis

Primary amyloidosis

Eosinophilic pneumonia

Diffuse alveolar hemorrhage

Pulmonary lymphoma

Alveolar microlithiasis

Systemic vasculitides

Lymphangioleiomyomatosis

Tuberous sclerosis

Neurofibromatosis

Adult respiratory distress syndrome

Acquired immunodeficiency syndrome

Bone marrow transplantation

Hermansky-Pudlack syndrome

Systemic lipoidosis (Gaucher's, Niemann-Pick)

Alveolar cell carcinoma

Respiratory bronchiolitis

Drug Or Treatment Induced

Antibiotics (furantoin, sulfasalazine)

Anti-inflammatory agents (aspirin, gold, penicillamine)

Antiarrhythmics (amiodarone, tocainide)

Chemotherapeutic agents

Antibiotics (bleomycin, mitomycin)

Alkylating agents (busulfan, cyclophosphamide, chlorambucil)

Antimetabolites (methotrexate, azathioprine, cytosine arabinoside)

Nitrosureas (BCNU, CCNU)

Others (procarbazine, vinblastine)

Oxygen

Vitamins (tryptophan)

Radiation

Narcotics (heroin, crack cocaine, methadone, morphine)

Occupation or Environment Related

Hypersensitivity pneumonitis (> 50 entities)

Farmers' lung

Bird breeders' lung

Humidifier lung

Pneumoconiosis

Silicosis

Berylliosis

Asbestosis

Stannosis

Hard metal pneumoconiosis

Aluminum oxide fibrosis

Coal workers' pneumoconiosis

Siderosis

Talc pneumoconiosis

Adapted from: Schwarz MI. Clinical overview of the interstitial lung diseases. In: Schwarz MI, King TE, Jr, eds. Interstitial Lung Disease, 2nd ed. Philadelphia: Mosby Year Book, 1993.

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