Disorder | People Most Often Affected | Percentage of Affected People Who Smoke Cigarettes | Treatment | Outlook |
---|---|---|---|---|
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 Corticosteroids | 70% survival at 10 years. |
Nonspecific interstitial pneumonia | More frequently, women aged 40–60 | Fewer than 40% | Corticosteroids | Fewer than 50% die in 5 years. Mortality rate: widely variable, but generally better than idiopathic pulmonary fibrosis. |
Cryptogenic organizing pneumonia | People of any age, usually aged 40–50 | Fewer than 50% | Corticosteroids | Relapses are common 5-year survival rate is > 90% Death is rare. |
Respiratory bronchiolitis–associated interstitial lung disease | People aged 30–50 (slightly more men) | More than 90% | Smoking cessation Corticosteroids | Death is rare as long as people stop smoking. |
Acute interstitial pneumonia | People of any age | Unknown | Best treatment unknown Sometimes corticosteroids | More than 50% die in less than 6 months. |
Lymphoid interstitial pneumonia | Mostly women of any age | Unknown | Corticosteroids | The prognosis is difficult to predict, |
Idiopathic pleuroparenchymal fibroelastosis | People of any age (median age 57) | Usually non-smokers | Best treatment unknown (possibly corticosteroids) | 5-year survival rate is approximately 25 to 60%. |
* In decreasing order of frequency. |