Interstitial Pneumonia with Autoimmune Features (IPAF): Case Report

Abstract

IPAF groups individuals with ILD and other clinical, serologic, or pulmonary manifestations with an underlying systemic autoimmune condition, but do not meet current rheumatologic criteria for a CTD. ILD is a frequent clinical manifestation of CTDs; may appear in the context of a well known CTD but is often the first and only manifestation of an unknown CTD. Identifying an underlying CTD in patients presenting with initial interstitial involvement can be challenging; such evaluations can be optimized using a multidisciplinary approach. We present the case of three patients, of different presentation, evolution and treatment, all characterized to date as IPAF.