title: Clinical associations of autoantibodies to a p155/140 kDa doublet protein in juvenile dermatomyositis.
abstract: OBJECTIVE
  Myositis-specific autoantibodies (MSAs) may define homogeneous clinical subsets of adult patients with dermatomyositis (DM). Recently, there have been descriptions of novel autoantibodies in DM. This study was conducted to establish the clinical significance of anti-p155/140 autoantibodies in juvenile DM (JDM).
  
  
  METHODS
  The first 116 children recruited to the JDM National Registry and Repository (UK and Ireland) were studied. Comprehensive clinical features were recorded and sera screened for anti-p155/140 autoantibodies using radio-immunoprecipitation. Sera from adults with DM (n = 20), PM (n = 25), SSc (n = 150), SLE (n = 40) and healthy subjects (n = 50) were used for comparison. Immunodepletion experiments were used to establish whether the p155/140 kDa targets recognized by JDM sera were the same as adult DM sera.
  
  
  RESULTS
  Twenty-seven out of 116 (23%) JDM cases were positive for anti-p155/140 in comparison with 6/20 (30%) adults with DM. Immunodepletion confirmed that the 155/140 kDa proteins recognized by JDM and adult DM sera were the same targets. All other adult control sera were negative for anti-p155/140 autoantibodies. There was a higher frequency of males in the anti-p155/140-positive JDM group (P = 0.02). JDM patients with anti-p155/140 autoantibodies had significantly more cutaneous involvement including Gottron's papules (P = 0.003), ulceration (P = 0.005) and oedema (P = 0.013). The distribution of skin lesions was more extensive particularly periorbitally (P = 0.014) and over the small (P < 0.001) and large joints (P = 0.003).
  
  
  CONCLUSIONS
  Anti-p155/140 autoantibodies are clinically significant in JDM and may define a clinical subset in terms of disease severity and outcome. The same autoantigen target is detected in adult DM patients.
authors.affiliation: Royal National Hospital for Rheumatic Diseases, Upper Borough Walls, Bath BA1 1RL, UK.
authors.forename: H
authors.initials: H
authors.lastname: Gunawardena
authors.forename: L R
authors.initials: LR
authors.lastname: Wedderburn
authors.forename: J
authors.initials: J
authors.lastname: North
authors.forename: Z
authors.initials: Z
authors.lastname: Betteridge
authors.forename: J
authors.initials: J
authors.lastname: Dunphy
authors.forename: H
authors.initials: H
authors.lastname: Chinoy
authors.forename: J E
authors.initials: JE
authors.lastname: Davidson
authors.forename: R G
authors.initials: RG
authors.lastname: Cooper
authors.forename: N J
authors.initials: NJ
authors.lastname: McHugh
mesh_terms: D000328:Adult; D017677:Age Distribution; D017668:Age of Onset; D000918:Antibody Specificity; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003882:Dermatomyositis / Q000453:epidemiology* / Q000276:immunology* / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D008297:Male; D009687:Nuclear Proteins / Q000097:blood / Q000276:immunology*; D011336:Probability; D011446:Prospective Studies; D012042:Registries; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D017678:Sex Distribution; D018709:Statistics, Nonparametric; D006113:United Kingdom / Q000453:epidemiology
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D009687:Nuclear Proteins