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Review
. 2019 May;16(5):525-533.
doi: 10.1513/AnnalsATS.201808-565CME.

Interstitial Pneumonia with Autoimmune Features

Bridget A Graney  1 , Aryeh Fischer  1   2
Affiliations
Review

Interstitial Pneumonia with Autoimmune Features

Bridget A Graney et al. Ann Am Thorac Soc. 2019 May.

Abstract

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) who 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 who fulfill IPAF criteria, suggestions for modification of the criteria have been offered, and patients who fulfill IPAF criteria are being included as a subset in the ongoing phase II multicenter unclassifiable ILD treatment trial with pirfenidone. 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. Clinical trial registered with www.clinicaltrials.gov (NCT03099187).

Keywords: connective tissue diseases; idiopathic interstitial pneumonias; interstitial lung diseases; pulmonary fibrosis.

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Figures

Figure 1.
Figure 1.
Classification of interstitial lung diseases. AIP = acute interstitial pneumonia; COP = cryptogenic organizing pneumonia; CTD = connective tissue disease; DIP = desquamative interstitial pneumonia; MCTD = mixed connective tissue disease; NSIP = nonspecific interstitial pneumonia; RA = rheumatoid arthritis; RB-ILD = respiratory bronchiolitis–associated interstitial lung disease; SLE = systemic lupus erythematosus. Adapted by permission from Reference .
Figure 2.
Figure 2.
Continuum on which interstitial pneumonia with autoimmune features (IPAF) sits between the idiopathic interstitial pneumonias and defined connective tissue disease–related interstitial lung diseases (CTD-ILDs). IIP = idiopathic interstitial pneumonia.
See this image and copyright information in PMC

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