Table 1.

Types of interstitial lung disease (ILD) usually or potentially associated with progressive fibrosis

1. Idiopathic interstitial pneumonia

• Idiopathic pulmonary fibrosis (ie, idiopathic usual interstitial pneumonia)

• Nonspecific interstitial pneumonia (NSIP)

• Desquamative interstitial pneumonia (DIP)

• Respiratory bronchiolitis-associated interstitial lung disease (RBILD)

• Acute interstitial pneumonia (AIP)

• Cryptogenic organizing pneumonia (COP)

• Lymphocytic interstitial pneumonia (idiopathic)

• Pleuroparenchymal fibroelastosis (idiopathic)

• Nonclassifiable interstitial pneumonia (NCIP)

2. Connective tissue disease associated

• Rheumatoid arthritis

• Systemic sclerosis (scleroderma)

• Antisynthetase syndromes

• Sjögren syndrome

• Systemic lupus erythematosus

• Ankylosing spondylitis

• Interstitial pneumonia with autoimmune features

3. Chronic hypersensitivity pneumonitis

4. Primary disease related

• Sarcoidosis

• Pulmonary Langerhans cell histiocytosis (PLCH)

• Eosinophilic lung disease related (eg, eosinophilic pneumonia)

• Chronic aspiration

5. Iatrogenic

• Drug induced

• Radiation pneumonitis/fibrosis

6. Pneumoconioses

7. Inherited lung disease

• Familial interstitial pneumonia (FIP)

• Hermansky–Pudlak syndrome (HPS)

• Other (eg, metabolic storage diseases)

8. Miscellaneous disorders

• Interstitial pneumonia with autoimmune features (IPAF)

• Acute fibrinous and organizing pneumonia (AFOP)

• Bronchiolocentric pattern of interstitial pneumonia

9. Nonclassifiable ILD