Comparing Types of Idiopathic Interstitial Pneumonias*

Disorder

People Most Often Affected

Percentage of Affected People Who Smoke Cigarettes

Treatment

Outlook

Idiopathic pulmonary fibrosis

More frequently, men over 50 years

More than 60%

Pulmonary rehabilitation

Pirfenidone or nintedanib Pirfenidone or nintedanib

Lung transplantation

50–70% die in 5 years

Desquamative interstitial pneumonia

More frequently, men aged 30–50 years

More than 90%

Smoking cessation

Steroids

70% survival at 10 years

Nonspecific interstitial pneumonia

More frequently, women aged 40–60 years

Fewer than 30%

Steroids

Sometimes other immunosuppressants

F

Mortality rate varies widely, but generally better than idiopathic pulmonary fibrosis

Cryptogenic organizing pneumonia

People of any age, usually aged 50–60 years

Fewer than 50%

Steroids

5-year survival rate is > 90%

Respiratory bronchiolitis–associated interstitial lung disease

People aged 30–50 years (slightly more men)

More than 90%

Smoking cessation

Steroids

Death is rare as long as people stop smoking

Acute interstitial pneumonia

People of any age

Unknown

Mechanical ventilation (machine-assisted breathing)

Sometimes steroids

More than 50% die in less than 6 months

Lymphoid interstitial pneumonia

Mostly women of any age

Unknown

Steroids

The prognosis is difficult to predict

Idiopathic pleuroparenchymal fibroelastosis

People of any age (median age 57 years)

Usually people who do not smoke

Best treatment unknown (possibly steroids)

5-year survival rate is approximately 25 to 60%

* In decreasing order of frequency.