TY - JOUR T1 - Myositis-specific Antibodies Identify A Distinct Interstitial Pneumonia with Autoimmune Features Phenotype JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.01205-2020 SP - 2001205 AU - Julia Graham AU - Iazsmin Bauer Ventura AU - Chad A. Newton AU - Cathryn Lee AU - Noelle Boctor AU - Janelle Vu Pugashetti AU - Claire Cutting AU - Elena Joerns AU - Habrinder Sandhu AU - Jonathan H. Chung AU - Christine Kim Garcia AU - Michael Kadoch AU - Imre Noth AU - Ayodeji Adegunsoye AU - Mary E. Strek AU - Justin M. Oldham Y1 - 2020/01/01 UR - http://erj.ersjournals.com/content/early/2020/07/02/13993003.01205-2020.abstract N2 - 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.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Graham has nothing to disclose.Conflict of interest: Dr. Bauer Ventura has nothing to disclose.Conflict of interest: Dr. Newton has nothing to disclose.Conflict of interest: Dr. Lee has nothing to disclose.Conflict of interest: Dr. Boctor has nothing to disclose.Conflict of interest: Dr. Vu Pugashetti has nothing to disclose.Conflict of interest: Dr. Cutting has nothing to disclose.Conflict of interest: Dr. Joerns reports grants from Pfizer, outside the submitted work.Conflict of interest: Dr. Sandhu has nothing to disclose.Conflict of interest: Dr. Chung has nothing to disclose.Conflict of interest: Dr. Kadoch has nothing to disclose.Conflict of interest: Dr. Noth reports personal fees from BI, personal fees from Genentech, grants from NIH, grants from NIH , grants from NIH, personal fees from Gilead/perceptive, grants from HLR, grants from Stromedix, grants from Promedior, personal fees from PILOT CME, personal fees from Sunovion, outside the submitted work; In addition, Dr. Noth has a patent TOLLIP in IPF pending, a patent Plasma proteins in IPF issued, and a patent PBMC expression signature in IPF pending.Conflict of interest: Dr. Adegunsoye reports other from Boehringer Ingelheim, grants from Pulmonary Fibrosis Foundation, grants from The CHEST Foundation, outside the submitted work.Conflict of interest: Dr. Strek reports grants from Boehringer Ingelheim, grants from Novartis, personal fees and non-financial support from Boehringer Ingelheim, outside the submitted work.Conflict of interest: Dr. Oldham reports grants from National Institutes of Health, grants from American College of Chest Physicians, during the conduct of the study; personal fees from Boehringer Ingelheim, personal fees from Genentech, outside the submitted work.Conflict of interest: Dr. Garcia reports grants from NIH, during the conduct of the study; grants from Astra Zeneca, outside the submitted work. ER -