title: Clinical features and outcomes of interstitial lung disease in anti-Jo-1 positive antisynthetase syndrome.
abstract: Interstitial lung disease (ILD) is a common extra-muscular manifestation of antisynthetase (AS) syndrome. ILD prevalence is higher with anti-Jo-1 antibody positivity. Data on long-term outcomes in these patients are lacking.
  
  
  
  Over 15 years, we identified subjects with anti-Jo-1 positive AS syndrome and ILD. Demographics, pulmonary function testing (PFT), high-resolution computed tomography (HRCT), histopathology, and long-term survival were analyzed.
  
  
  
  We identified 103 subjects (mean age 49.2 years, female predominance [70%]). The predominant myopathy was polymyositis (64%) followed by dermatomyositis (24%). In approximately half of studied subjects, AS syndrome and ILD were diagnosed within 6 months of each other. The majority had restriction on PFTs (98%). Non-specific interstitial pneumonia (NSIP) was the most common HRCT pattern (52%), followed by NSIP overlapping with organizing pneumonia (OP) (22%). Thirty-nine subjects had biopsy data. Ten-year survival was 68%. Multivariable analysis adjusted for age at ILD diagnosis, gender, FVC and DLCO, revealed that male gender (HR = 2.60, p = 0.04) and DLCO at presentation (HR = 0.94, p = 0.05) significantly predicted mortality.
  
  
  
  We present a large cohort of anti-Jo-1 positive AS syndrome with ILD and note good overall survival.
authors: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
authors: Ana C
authors: AC
authors: Zamora
authors: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
authors: Sumedh S
authors: SS
authors: Hoskote
authors: Division of Pulmonary Medicine, Instituto de Investigacion Sanitaria Valdecilla (IDIVAL), Santander, Spain.
authors: Beatriz
authors: B
authors: Abascal-Bolado
authors: Department of Radiology, Mayo Clinic, Rochester, MN, USA.
authors: Darin
authors: D
authors: White
authors: Department of Radiology, Mayo Clinic, Rochester, MN, USA.
authors: Christian W
authors: CW
authors: Cox
authors: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
authors: Jay H
authors: JH
authors: Ryu
authors: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA. Electronic address: moua.teng@mayo.edu.
authors: Teng
authors: T
authors: Moua
mesh_terms: D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000276:immunology*; D015331:Cohort Studies; D003882:Dermatomyositis / Q000453:epidemiology / Q000276:immunology; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D017063:Outcome Assessment, Health Care; D017285:Polymyositis / Q000453:epidemiology / Q000276:immunology; D015995:Prevalence; D012129:Respiratory Function Tests / Q000379:methods; D012189:Retrospective Studies; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed
chemical_list: D000974:Antibodies, Antinuclear; C046750:Jo-1 antibody
keywords: Antinuclear antibody; Antisynthetase syndrome; Dermatomyositis; Interstitial lung disease; Jo-1 antibody; Polymyositis