Treatment and Prognosis of Idiopathic Interstitial Pneumonias*

Disorder

Treatment

Prognosis

Idiopathic pulmonary fibrosis

Lung transplantation

Mortality rate: 50–70% in 5 years

Desquamative interstitial pneumonia

Smoking cessation

70% survival at 10 years

Nonspecific interstitial pneumonia

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

Cryptogenic organizing pneumonia

Corticosteroids

5-year survival rate is > 90%

Respiratory bronchiolitis–associated interstitial lung disease

Smoking cessation

Mortality rate: Rare with smoking cessation

Acute interstitial pneumonia

Supportive care; often corticosteroids

Mortality rate: > 50% in < 6 months

Lymphocytic interstitial pneumonia

Corticosteroids

Not well defined

Idiopathic pleuroparenchymal fibroelastosis

Appropriate treatment unknown; often corticosteroids

5-year survival rates approximately 25 to 60%

* Listed in order of decreasing frequency.