PMID- 30695649
OWN - NLM
STAT- MEDLINE
DCOM- 20200518
LR  - 20200518
IS  - 2325-6621 (Electronic)
IS  - 2329-6933 (Print)
IS  - 2325-6621 (Linking)
VI  - 16
IP  - 5
DP  - 2019 May
TI  - Interstitial Pneumonia with Autoimmune Features.
PG  - 525-533
LID - 10.1513/AnnalsATS.201808-565CME [doi]
AB  - 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).
FAU - Graney, Bridget A
AU  - Graney BA
AD  - 1 Division of Pulmonary Sciences and Critical Care Medicine and.
FAU - Fischer, Aryeh
AU  - Fischer A
AD  - 1 Division of Pulmonary Sciences and Critical Care Medicine and.
AD  - 2 Division of Rheumatology, University of Colorado Anschutz Medical Campus, 
      Aurora, Colorado.
LA  - eng
SI  - ClinicalTrials.gov/NCT03099187
GR  - T32 HL007085/HL/NHLBI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - United States
TA  - Ann Am Thorac Soc
JT  - Annals of the American Thoracic Society
JID - 101600811
SB  - IM
MH  - Autoimmune Diseases/diagnosis/*immunology
MH  - *Autoimmunity
MH  - Humans
MH  - Lung Diseases, Interstitial/diagnosis/*immunology
PMC - PMC6850782
OTO - NOTNLM
OT  - connective tissue diseases
OT  - idiopathic interstitial pneumonias
OT  - interstitial lung diseases
OT  - pulmonary fibrosis
EDAT- 2019/01/30 06:00
MHDA- 2020/05/19 06:00
PMCR- 2020/05/01
CRDT- 2019/01/30 06:00
PHST- 2019/01/30 06:00 [pubmed]
PHST- 2020/05/19 06:00 [medline]
PHST- 2019/01/30 06:00 [entrez]
PHST- 2020/05/01 00:00 [pmc-release]
AID - 10.1513/AnnalsATS.201808-565CME [doi]
PST - ppublish
SO  - Ann Am Thorac Soc. 2019 May;16(5):525-533. doi: 10.1513/AnnalsATS.201808-565CME.