Idiopathic interstitial pneumonias: review of the latest American Thoracic Society/European Respiratory Society classification

Abstract The diagnosis of idiopathic interstitial pneumonias (IIPs) involves a multidisciplinary scenario in which the radiologist assumes a key role. The latest (2013) update of the IIP classification by the American Thoracic Society/European Respiratory Society proposed some important changes to the original classification of 2002. The novelties include the addition of a new disease (idiopathic pleuroparenchymal fibroelastosis) and the subdivision of the IIPs into four main groups: chronic fibrosing IIPs (idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia); smoking-related IIPs (desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease); acute or subacute IIPs (cryptogenic organizing pneumonia and acute interstitial pneumonia); rare IIPs (lymphoid interstitial pneumonia and idiopathic pleuroparenchymal fibroelastosis); and the so-called “unclassifiable” IIPs. In this study, we review the main clinical, tomographic, and pathological characteristics of each IIP.