Interstitial Pneumonia with Autoimmune Features: 8 Years after Nomenclature and Classification —Where are We Not and Where Are We Headed?

AbstractPurpose of ReviewThe term interstitial pneumonia with autoimmune features (IPAF) was proposed by an international task force as a research classification to standardize nomenclature for patients with idiopathic interstitial pneumonia (IIP) and features of connective tissue disease (CTD). This review aims to discuss the advances made in understanding this research classification and the clinical implications of this term.Recent FindingsMultiple cohort studies have described the clinical presentation of patients meeting criteria for IPAF with heterogeneity noted particularly in the morphologic domain as compared to clinical and serologic domains. Treating these patients according to their clinical presentation and features remains the key expert opinion given the paucity of data to inform therapeutic strategies in IPAF.SummaryThere are still several challenges and unresolved questions which preclude the application of IPAF as a clinical diagnosis. Whether IPAF represents a clinical diagnosis distinct from CTD-ILD or IIPs or precursor or an overlap of these conditions remains controversial and warrants further investigation.
Source: Current Respiratory Care Reports - Category: Respiratory Medicine Source Type: research