Current concepts and dilemmas in idiopathic interstitial pneumonias

Abstract

Idiopathic interstitial pneumonias comprise approximately one-third of interstitial lung diseases (also called diffuse parenchymal infiltrative lung diseases). The classification of idiopathic interstitial pneumonias has undergone several revisions since the initial description of 40 years ago, and the most recent version was published in 2013. Although some aspects have been clarified, this group of heterogeneous disorders continues to be a source of confusion and misunderstanding in clinical applications. In this article, we explore several topical themes in the evaluation and management of patients with idiopathic interstitial pneumonias.