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Ann Am Thorac Soc. 2019 Jan 29. doi: 10.1513/AnnalsATS.201808-565CME. [Epub ahead of print]

Interstitial Pneumonia with Autoimmune Features.

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University of Colorado at Denver - Anschutz Medical Campus, 129263, Division of Pulmonary Sciences and Critical Care Medicine, Aurora, Colorado, United States.
University of Colorado Denver School of Medicine, 12225, Medicine, Aurora, Colorado, United States ;


"Interstitial pneumonia with autoimmune features" (IPAF) is a research classification proposed by the European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease as an initial step to uniformly define, identify and study patients with interstitial lung disease (ILD) that have features of autoimmunity, yet fall short of a characterizable connective tissue disease. Since its publication in July 2015, there has been substantial interest in IPAF. Centers from around the world have published their findings of retrospectively identified cohorts of patients that fulfill IPAF criteria, suggestions for modification of the criteria have been offered and patients that fulfill IPAF criteria are being included as a subset in the ongoing phase II multicenter unclassifiable ILD treatment trial with pirfenidone ( identifier: NCT03099187). The IPAF designation represents an important first step toward studying and furthering our understanding of the natural history of this cohort of patients with ILD using uniform nomenclature and a standardized set of criteria. Prospective evaluations and, ideally, interdisciplinary and multicenter collaborations will inform best practices for treatment and management and will guide future refinement to the IPAF criteria. This review focuses on the relevant background that led to the development of IPAF, summarizes the proposed criteria, discusses cohort studies of patients with IPAF published to date and what they have taught us about the IPAF phenotype, and offers insights into future directions in this arena.

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