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. | ||||