Myositis-specific Antibodies Identify A Distinct Interstitial Pneumonia with Autoimmune Features Phenotype.

Autor:	Graham J; Department of Internal Medicine, University of California at Davis.; These authors contributed equally., Bauer Ventura I; Department of Medicine, Section of Rheumatology, University of Chicago.; These authors contributed equally., Newton CA; Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, University of Texas Southwestern.; These authors contributed equally., Lee C; Department of Medicine, Section of Pulmonary and Critical Care, University of Chicago., Boctor N; Department of Internal Medicine, University of California at Davis., Pugashetti JV; Department of Internal Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of California at Davis., Cutting C; Department of Internal Medicine, University of California at Davis., Joerns E; Department of Internal Medicine, Division of Rheumatology, University of Texas Southwestern., Sandhu H; Department of Internal Medicine, Division of Rheumatology, University of California at Davis., Chung JH; Department of Radiology, The University of Chicago., Garcia CK; Department of Medicine, Division of Pulmonary, Allergy and Critical Care Medicine, Columbia University., Kadoch M; Department of Radiology, University of California at Davis., Noth I; Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Virginia., Adegunsoye A; Department of Medicine, Section of Pulmonary and Critical Care, University of Chicago., Strek ME; Department of Medicine, Section of Pulmonary and Critical Care, University of Chicago.; These authors contributed equally., Oldham JM; Department of Internal Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of California at Davis joldham@ucdavis.edu.; These authors contributed equally.
Jazyk:	angličtina
Zdroj:	The European respiratory journal [Eur Respir J] 2020 Jul 16. Date of Electronic Publication: 2020 Jul 16.
DOI:	10.1183/13993003.01205-2020
Abstrakt:	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. (Copyright ©ERS 2020.)
Databáze:	MEDLINE
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