PMID- 32675203
OWN - NLM
STAT- Publisher
LR  - 20240227
IS  - 1399-3003 (Electronic)
IS  - 0903-1936 (Print)
IS  - 0903-1936 (Linking)
DP  - 2020 Jul 16
TI  - Myositis-specific Antibodies Identify A Distinct Interstitial Pneumonia with 
      Autoimmune Features Phenotype.
LID - 10.1183/13993003.01205-2020 [doi]
LID - 2001205
AB  - Interstitial pneumonia with autoimmune features (IPAF) characterises individuals 
      with interstitial lung disease (ILD) and features of connective tissue disease 
      (CTD) who fail to satisfy CTD criteria. Inclusion of myositis-specific antibodies 
      (MSAs) in the IPAF criteria has generated controversy, as these patients also 
      meet proposed criteria for an anti-synthetase syndrome. Whether MSAs and myositis 
      associated antibodies (MAA) identify phenotypically distinct IPAF subgroups 
      remains unclear.A multi-center, retrospective investigation was conducted to 
      assess clinical features and outcomes in patients meeting IPAF criteria 
      stratified by the presence of MSAs and MAAs. IPAF subgroups were compared to 
      cohorts of patients with idiopathic inflammatory myopathy-ILD (IIM-ILD), 
      idiopathic pulmonary fibrosis (IPF) and non-IIM CTD-ILDs. The primary endpoint 
      assessed was three-year transplant-free survival. Two hundred sixty-nine patients 
      met IPAF criteria, including 35 (13%) with MSAs and 65 (24.2%) with MAAs. 
      Survival was highest among patients with IPAF-MSA and closely approximated those 
      with IIM-ILD. Survival did not differ between IPAF-MAA and IPAF without MSA/MAA 
      cohorts. Usual interstitial pneumonia (UIP) morphology was associated with 
      differential outcome risk, with IPAF patients with non-UIP morphology 
      approximating survival observed in non-IIM CTD-ILDs. MSAs, but not MAAs 
      identified a unique IPAF phenotype characterised by clinical features and 
      outcomes similar to IIM-ILD. UIP morphology was a strong predictor of outcome in 
      others meeting IPAF criteria. Because IPAF is a research classification without 
      clear treatment approach, these findings suggest MSAs should be removed from the 
      IPAF criteria and such patients should be managed as an IIM-ILD.
CI  - Copyright (c)ERS 2020.
FAU - Graham, Julia
AU  - Graham J
AD  - Department of Internal Medicine, University of California at Davis.
AD  - These authors contributed equally.
FAU - Bauer Ventura, Iazsmin
AU  - Bauer Ventura I
AD  - Department of Medicine, Section of Rheumatology, University of Chicago.
AD  - These authors contributed equally.
FAU - Newton, Chad A
AU  - Newton CA
AD  - Department of Internal Medicine, Division of Pulmonary and Critical Care 
      Medicine, University of Texas Southwestern.
AD  - These authors contributed equally.
FAU - Lee, Cathryn
AU  - Lee C
AD  - Department of Medicine, Section of Pulmonary and Critical Care, University of 
      Chicago.
FAU - Boctor, Noelle
AU  - Boctor N
AD  - Department of Internal Medicine, University of California at Davis.
FAU - Pugashetti, Janelle Vu
AU  - Pugashetti JV
AD  - Department of Internal Medicine, Division of Pulmonary, Critical Care and Sleep 
      Medicine, University of California at Davis.
FAU - Cutting, Claire
AU  - Cutting C
AD  - Department of Internal Medicine, University of California at Davis.
FAU - Joerns, Elena
AU  - Joerns E
AD  - Department of Internal Medicine, Division of Rheumatology, University of Texas 
      Southwestern.
FAU - Sandhu, Habrinder
AU  - Sandhu H
AD  - Department of Internal Medicine, Division of Rheumatology, University of 
      California at Davis.
FAU - Chung, Jonathan H
AU  - Chung JH
AD  - Department of Radiology, The University of Chicago.
FAU - Garcia, Christine Kim
AU  - Garcia CK
AD  - Department of Medicine, Division of Pulmonary, Allergy and Critical Care 
      Medicine, Columbia University.
FAU - Kadoch, Michael
AU  - Kadoch M
AD  - Department of Radiology, University of California at Davis.
FAU - Noth, Imre
AU  - Noth I
AD  - Department of Medicine, Division of Pulmonary and Critical Care Medicine, 
      University of Virginia.
FAU - Adegunsoye, Ayodeji
AU  - Adegunsoye A
AD  - Department of Medicine, Section of Pulmonary and Critical Care, University of 
      Chicago.
FAU - Strek, Mary E
AU  - Strek ME
AD  - Department of Medicine, Section of Pulmonary and Critical Care, University of 
      Chicago.
AD  - These authors contributed equally.
FAU - Oldham, Justin M
AU  - Oldham JM
AD  - Department of Internal Medicine, Division of Pulmonary, Critical Care and Sleep 
      Medicine, University of California at Davis joldham@ucdavis.edu.
AD  - These authors contributed equally.
LA  - eng
GR  - K23 HL146942/HL/NHLBI NIH HHS/United States
GR  - T32 HL007605/HL/NHLBI NIH HHS/United States
GR  - R01 HL093096/HL/NHLBI NIH HHS/United States
GR  - R01 HL130796/HL/NHLBI NIH HHS/United States
GR  - K23 HL148498/HL/NHLBI NIH HHS/United States
GR  - K23 HL138190/HL/NHLBI NIH HHS/United States
PT  - Journal Article
DEP - 20200716
PL  - England
TA  - Eur Respir J
JT  - The European respiratory journal
JID - 8803460
SB  - IM
PMC - PMC7943372
MID - NIHMS1673547
EDAT- 2020/07/18 06:00
MHDA- 2020/07/18 06:00
PMCR- 2022/01/16
CRDT- 2020/07/18 06:00
PHST- 2020/04/15 00:00 [received]
PHST- 2020/06/28 00:00 [accepted]
PHST- 2020/07/18 06:00 [entrez]
PHST- 2020/07/18 06:00 [pubmed]
PHST- 2020/07/18 06:00 [medline]
PHST- 2022/01/16 00:00 [pmc-release]
AID - 13993003.01205-2020 [pii]
AID - 10.1183/13993003.01205-2020 [doi]
PST - aheadofprint
SO  - Eur Respir J. 2020 Jul 16:2001205. doi: 10.1183/13993003.01205-2020.