Causes of Interstitial Lung Disease

Category

Examples

Systemic rheumatic disorders

Ankylosing spondylitis (rare)

Autoimmune myositis

Behçet disease (very rare)

Goodpasture syndrome

IgG-4 related disease

Mixed connective tissue disease

Rheumatoid arthritis

Sjögren syndrome

Systemic lupus erythematosus

Systemic sclerosis

Medications and illicit drugs

Selected list:

Genetic disorders

Fabry disease (rare)

Familial pulmonary fibrosis

Gaucher disease (rare)

Hermansky-Pudlak Syndrome

Neurofibromatosis

Niemann-Pick disease (rare)

Pulmonary alveolar microlithiasis

Pulmonary alveolar proteinosis

Tuberous sclerosis

Idiopathic interstitial pneumonias

Acute interstitial pneumonia

Cryptogenic organizing pneumonia

Desquamative interstitial pneumonia

Idiopathic pleuroparenchymal fibroelastosis

Idiopathic pulmonary fibrosis

Lymphocytic interstitial pneumonia

Nonspecific interstitial pneumonia

Respiratory bronchiolitis–associated interstitial lung disease

Occupational and environmental exposure

Inorganic (selected): Aluminosis (caused by exposure to metallic aluminum powder), asbestosis, baritosis, beryllium disease, coal workers’ pneumoconiosis, exposure to hard metals (eg, cadmium, cobalt, titanium oxide, tungsten, vanadium carbides), radiation fibrosis, siderosis, silicosis

Organic (selected): Bagassosis, bird fancier’s lung, coffee worker’s lung, farmer’s lung, hot tub lung, humidifier lung, malt worker’s lung, maple bark stripper’s lung, mushroom worker’s lung, tea grower’s lung (see Hypersensitivity Pneumonitis)

Vasculitis

Eosinophilic granulomatosis with polyangiitis

Giant cell arteritis (rare)

Granulomatosis with polyangiitis

Microscopic polyangiitis

Polyarteritis nodosa (rare)

Takayasu arteritis (rare)

Miscellaneous disorders

Amyloidosis

Chronic aspiration

Eosinophilic pneumonia, acute and chronic

Lipoid pneumonia

Lymphangioleiomyomatosis

Pulmonary Langerhans cell histiocytosis

Sarcoidosis