Treatment and Prognosis of Idiopathic Interstitial Pneumonias*

Idiopathic pulmonary fibrosis (IPF)

Pirfenidone or nintedanibPirfenidone or nintedanib

Lung transplantation

Mortality rate: 50–70% in 5 years

Desquamative interstitial pneumonia (DIP)

Smoking cessation

Glucocorticoids

70% survival at 10 years

Nonspecific interstitial pneumonia (NSIP)

Glucocorticoids with or without other immunosuppressive therapies (eg, azathioprine, mycophenolate mofetil)Glucocorticoids with or without other immunosuppressive therapies (eg, azathioprine, mycophenolate mofetil)

Mortality rate: widely variable, but generally better than idiopathic pulmonary fibrosis; in purely cellular disease (rare), extremely low

Cryptogenic organizing pneumonia (COP)

Glucocorticoids

5-year survival rate is > 90%

Respiratory bronchiolitis–associated interstitial lung disease (RBILD)

Smoking cessation

Glucocorticoids

Mortality rate: Rare with smoking cessation

Acute interstitial pneumonia (AIP)

Supportive care; mechanical ventilation; often glucocorticoids

Mortality rate: > 50% in < 6 months

Lymphoid interstitial pneumonia (LIP)

Glucocorticoids

Not well defined

Idiopathic pleuroparenchymal fibroelastosis (IPPFE)

Appropriate treatment unknown; often glucocorticoids

5-year survival rates approximately 25 to 60%