Challenges in idiopathic interstitial lung disease

Idiopathic interstitial pneumonias (IIPs) are a group of pulmonary disorders with distinct histologic and radiologic appearances and no identiiable cause. The new classiication of IIPs published in 2013 distinguishes six distinct major entities, including chronic, usually progressive ibrosing diseases, such as idiopathic pulmonary ibrosis (IPF) and idiopathic nonspeciic interstitial pneumonia. IPF, an invariably progressive and ultimately fatal lung disease that occurs in older adults, is the most frequent among the IIPs. Recent evidence and international guidelines advocate the importance of chest high-resolution computed tomography and multidisciplinary discussion (MDD) in the initial diagnostic assessment of patients with suspected IPF. MDD is currently considered the gold standard because improves the accuracy of IIPs diagnosis, avoiding unnecessary testing, and optimizing patient management, particularly nowadays that two drugs have been approved by regulatory agencies for the treatment of IPF. In this review, we focus on the revised diagnostic criteria for IIPs and IPF and provide an overview of the most recent clinical trials. Finally, we stress the fact that NSIP, one of the most frequent differential diagnosis in cases presenting with suspected IPF, is not anymore considered a provisional entity, but a deinite clinical-pathological entity.