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 VL - 56 IS - 6 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 - 2020/12/01 UR - http://erj.ersjournals.com/content/56/6/2001205.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 antisynthetase syndrome. Whether MSAs and myositis-associated antibodies (MAA) identify phenotypically distinct IPAF subgroups remains unclear.A multicentre, 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 and non-IIM CTD-ILDs. The primary end-point assessed was 3-year transplant-free survival.269 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 that MSAs should be removed from the IPAF criteria and such patients should be managed as an IIM-ILD.Myositis-specific antibodies identify a distinct interstitial pneumonia with autoimmune features phenotype characterised by clinical features and outcomes similar to patients with ILD due to an idiopathic inflammatory myopathy https://bit.ly/2VCmtab ER -