title: Clinical and pathologic differences in interstitial lung disease based on antisynthetase antibody type.
abstract: BACKGROUND
  Interstitial lung disease (ILD) is a common extramuscular manifestation of the idiopathic inflammatory myopathies (IIMs), dermatomyositis (DM) and polymyositis (PM). Patients with antisynthetase antibodies (ASA) demonstrate some or all of the features of the antisynthetase syndrome including IIM and ILD. It has been hypothesized that the clinical expression of antisynthetase syndrome varies between specific ASAs.
  
  
  OBJECTIVE
  We sought to determine whether the myositis-associated ILD (MA-ILD) phenotype differs based on the presence of ASAs and by ASA subtype.
  
  
  METHODS
  A cross-sectional and longitudinal analysis of consecutive patients enrolled at the Johns Hopkins Myositis Center with ILD in the setting of clinically diagnosed autoimmune myositis was conducted.
  
  
  RESULTS
  Seventy-seven subjects were included; 36 were ASA negative, 28 were anti-Jo1 positive, and 13 were non-Jo1 ASA positive (5 anti-PL-12, 4 anti-PL-7, 2 anti-EJ, and 2 anti-OJ). Non-Jo1 ASA positive participants were more likely to be African-American than Caucasian as compared to both the anti-Jo1 positive (p = 0.01) and ASA negative groups (p < 0.01). ASA negative participants had better mean forced vital capacity percent predicted (FVC%) and total computed tomography scores over time compared to those with anti-Jo1 after controlling for potential confounders.
  
  
  CONCLUSIONS
  ASA status was significantly different by race. Those with anti-Jo1 antibodies had worse lung function and CT scores over time compared to those without detectable antisynthetase antibodies. Further prospective study in a larger cohort is needed to determine whether these apparent antibody-specific differences in demographics and manifestations of disease translate into meaningful disparities in clinical outcomes.
authors.affiliation: Johns Hopkins University School of Medicine, Division of Pulmonary and Critical Care Medicine, 1830 E. Monument Street Suite 500, Baltimore, MD 21205, United States.
authors.forename: C
authors.initials: C
authors.lastname: Johnson
authors.affiliation: Yale University School of Medicine, Division of Pulmonary, Critical Care & Sleep, 15 York Street, LCI 105, New Haven, CT 06510, United States.
authors.forename: G R
authors.initials: GR
authors.lastname: Connors
authors.affiliation: Reston Radiology Consultants, PC, 1800 Town Center Parkway, Reston, VA 20190, United States.
authors.forename: J
authors.initials: J
authors.lastname: Oaks
authors.affiliation: University of Pittsburgh Medical Center, Division of Pulmonary, Allergy and Critical Care Medicine, 3459 Fifth Avenue, Pittsburgh, PA 15213, United States; National Institutes of Health, Critical Care Medicine Department, 10 Center Drive, Bethesda, MD 20892, United States.
authors.forename: S
authors.initials: S
authors.lastname: Han
authors.affiliation: Emory University Hospital, Emory University Hospital Midtown, 550 Peachtree St, Atlanta, GA 30308, United States.
authors.forename: A
authors.initials: A
authors.lastname: Truong
authors.affiliation: Johns Hopkins University School of Medicine, Division of Pulmonary and Critical Care Medicine, 1830 E. Monument Street Suite 500, Baltimore, MD 21205, United States.
authors.forename: B
authors.initials: B
authors.lastname: Richardson
authors.affiliation: Johns Hopkins University School of Medicine, Division of Pulmonary and Critical Care Medicine, 1830 E. Monument Street Suite 500, Baltimore, MD 21205, United States.
authors.forename: N
authors.initials: N
authors.lastname: Lechtzin
authors.affiliation: Johns Hopkins University School of Medicine, Department of Neurology, 4940 Eastern Avenue, Baltimore, MD 21224, United States; Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Expression, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD 20892, United States.
authors.forename: A L
authors.initials: AL
authors.lastname: Mammen
authors.affiliation: Johns Hopkins University School of Medicine, Division of Rheumatology, 4940 Eastern Avenue, Baltimore, MD 21224, United States.
authors.forename: L
authors.initials: L
authors.lastname: Casciola-Rosen
authors.affiliation: Johns Hopkins University School of Medicine, Division of Rheumatology, 4940 Eastern Avenue, Baltimore, MD 21224, United States.
authors.forename: L
authors.initials: L
authors.lastname: Christopher-Stine
authors.affiliation: Johns Hopkins University School of Medicine, Division of Pulmonary and Critical Care Medicine, 1830 E. Monument Street Suite 500, Baltimore, MD 21205, United States. Electronic address: sdanoff@jhmi.edu.
authors.forename: S K
authors.initials: SK
authors.lastname: Danoff
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000276:immunology*; D016022:Case-Control Studies; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000276:immunology*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014797:Vital Capacity
chemical_list: D001323:Autoantibodies
keywords: Antisynthetase syndrome; Dermatomyositis; ILD; Polymyositis