Лікування та прогноз ідіопатичної інтерстиціальної пневмонії*

Idiopathic pulmonary fibrosis (IPF)

Pirfenidone or nintedanib

Lung transplantation

Mortality rate: 50–70% in 5 years

Desquamative interstitial pneumonia (DIP)

Smoking cessation

Glucocorticoids

70% survival at 10 years

Nonspecific interstitial pneumonia (NSIP)

Glucocorticoids with or without other immunosuppressive therapies (eg, azathioprine, mycophenolate mofetil)

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

Cryptogenic organizing pneumonia (COP)

Glucocorticoids

5-year survival rate is > 90%

Respiratory bronchiolitis–associated interstitial lung disease (RBILD)

Smoking cessation

Glucocorticoids

Mortality rate: Rare with smoking cessation

Acute interstitial pneumonia (AIP)

Supportive care; mechanical ventilation; often glucocorticoids

Mortality rate: > 50% in < 6 months

Lymphoid interstitial pneumonia (LIP)

Glucocorticoids

Not well defined

Idiopathic pleuroparenchymal fibroelastosis (IPPFE)

Appropriate treatment unknown; often glucocorticoids

5-year survival rates approximately 25 to 60%