Merck Manual

Please confirm that you are not located inside the Russian Federation

honeypot link
Comparing Types Of Idiopathic Interstitial Pneumonias*

Comparing Types Of Idiopathic Interstitial Pneumonias*


People Most Often Affected

Percentage of Affected People Who Smoke Cigarettes



Idiopathic pulmonary fibrosis

More frequently, men over 50

More than 60%

Pulmonary rehabilitation

Lung transplantation

Pirfenidone or nintedanib

50–70% die in 5 years.

Desquamative interstitial pneumonia

More frequently, men aged 30–50

More than 90%

Smoking cessation


5% die in 5 years.

Nonspecific interstitial pneumonia

More frequently, women aged 40–60

Fewer than 40%


Fewer than 50% die in 5 years.

Cryptogenic organizing pneumonia

People of any age, usually aged 40–50

Fewer than 50%


Two thirds completely recover, but the disorder recurs in many.

Death is rare.

Respiratory bronchiolitis–associated interstitial lung disease

People aged 30–50 (slightly more men)

More than 90%

Smoking cessation


Death is rare.

Acute interstitial pneumonia

People of any age


Best treatment unknown

60% die in less than 6 months.

Lymphoid interstitial pneumonia

Mostly women of any age



The prognosis is difficult to predict.

Idiopathic pleuroparenchymal fibroelastosis

People of any age (median age 57)

Usually non-smokers

Best treatment unknown (possibly corticosteroids)

Disease progresses in 60% of people.

* In decreasing order of frequency.