Interstitial Pneumonia with Autoimmune Features: Aiming to Define, Refine, and Treat

Interstitial pneumonia with autoimmune features (IPAF) was defined for research purposes as interstitial lung disease (ILD) associated with features of autoimmunity without diagnosed rheumatic disease. Since publication of the IPAF criteria in 2015, there have been multiple studies of IPAF. However, much remains unknown regarding pathogenesis, prognosis, and treatment in IPAF. This narrative review details the history and classification of IPAF, lists challenges associated with classifying patients as IPAF, and explores the prevalence, epidemiology and presentation of IPAF. We also examine prognosis and important features determining IPAF clinical course, outline pathogenesis, and review treatment strategies.