Idiopathic interstitial pneumonias

Abstract

Idiopathic interstitial pneumonias represent approximately 30% of all interstitial lung diseases. The new classification of idiopathic interstitial pneumonias published in 2013 distinguishes 6 major entities, including chronic fibrosing forms (idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia), acute/subacute forms (cryptogenic organizing pneumonia and acute interstitial pneumonia) and smoking-related disorders (respiratory bronchiolitis interstitial lung disease and desquamative interstitial pneumonia). Pleuroparenchymal fibroelastosis is individualized as a new rare clinco-pathologic entity. For cases not fitting any spécifie clinicpathological category, a pragmatic classification based on disease behavior is proposed.