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pmid: 30695649
pmc: PMC6850782
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).
Humans, Autoimmunity, Lung Diseases, Interstitial, Autoimmune Diseases
Humans, Autoimmunity, Lung Diseases, Interstitial, Autoimmune Diseases
citations This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically). | 73 | |
popularity This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network. | Top 1% | |
influence This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically). | Top 10% | |
impulse This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network. | Top 1% |