Idiopathic Interstitial Pneumonias: A Radiology-Pathology Correlation Based on the Revised 2013 American Thoracic Society-European Respiratory Society Classification System

Abstract

The idiopathic interstitial pneumonias (IIPs) are a group of diffuse lung diseases that share many similar radiologic and pathologic features. According to the revised 2013 American Thoracic Society-European Respiratory Society classification system, these entities are now divided into major IIPs (idiopathic pulmonary fibrosis, idiopathic nonspecific interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, cryptogenic organizing pneumonia, and acute interstitial pneumonia), rare IIPs (idiopathic lymphoid interstitial pneumonia, idiopathic pleuroparenchymal fibroelastosis), and unclassifiable idiopathic interstitial pneumonias. Some of the encountered radiologic and histologic patterns can also be seen in the setting of other disorders, which makes them a diagnostic challenge. As such, the accurate classification of IIPs remains complex and is best approached through a collaboration among clinicians, radiologists, and pathologists, as the treatment and prognosis of these conditions vary greatly.