Idiopathic interstitial pneumonias

Abstract

This overview examines the history, progress, and present problems of idiopathic interstitial pneumonias (IIPs), mainly from a pathological viewpoint. The definition and classification of IIPs were first described by Liebow in the late 1960s, and were established in 2002 by an ATS/ERS international multidisciplinary consensus statement. This statement was then updated in 2013. The previous Japanese classification was incorporated with the international classification in 2003. Subsequently, remarkable progress has been achieved in the diagnosis and treatment of IIPs. The updated classification of 2013 included pleuroparenchymal fibroelastosis, unclassifiable interstitial pneumonia (IP), and classification according to disease behavior, which is related to the nature of and response to treatment of each disease. Antifibrotic drugs for idiopathic pulmonary fibrosis (IPF) have been effective for the improvement of pulmonary function. In the near future, genetic and molecular analysis of IIPs will provide information on the pathogenesis and prognosis of each disease.