title: Recent advances in dermatomyositis-specific autoantibodies.
abstract: OBJECTIVE
  In dermatomyositis, disease-specific autoantibodies now cover more than 70% of patients. These autoantibodies closely correlate with distinct clinical manifestations. In the past few years, extensive evidence has been accumulated on clinical significance of dermatomyositis-specific autoantibodies including autoantibodies against melanoma differentiation antigen 5 (MDA5), transcriptional intermediary factor 1 (TIF1), nuclear matrix protein 2 (NXP2), and small ubiquitin-like modifier activating enzyme (SAE).
  
  
  RESULTS
  Anti-MDA5 antibodies are found with high specificity in clinically amyopathic dermatomyositis presenting rapidly progressive interstitial lung disease (ILD) especially in Asian population. Similar tendency has been reported in the US/Europe, although the frequency of positivity and the type of ILD may differ. Anti-TIF1 antibodies are present in juvenile and adult dermatomyositis patients with close correlation with malignancy in adult population. Anti-NXP2 antibodies share similar phenotype with anti-TIF1 antibodies, except that anti-NXP2 antibodies are associated with calcinosis and severe muscle disease. Although numbers are still small, patients with anti-SAE antibodies tend to present skin disease first and then progress to muscle weakness with systematic symptoms including dysphagia. Moreover, distinct cutaneous manifestations and muscle histopathology findings for each autoantibody have been reported.
  
  
  CONCLUSIONS
  'Autoantibody-based classification' of dermatomyositis subsets is now a useful strategy for comprehending the heterogeneous spectrum of dermatomyositis.
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
authors.forename: Manabu
authors.initials: M
authors.lastname: Fujimoto
authors.forename: Rei
authors.initials: R
authors.lastname: Watanabe
authors.forename: Yosuke
authors.initials: Y
authors.lastname: Ishitsuka
authors.forename: Naoko
authors.initials: N
authors.lastname: Okiyama
mesh_terms: D001323:Autoantibodies / Q000032:analysis* / Q000276:immunology; D015415:Biomarkers / Q000032:analysis; D002114:Calcinosis / Q000276:immunology; D004268:DNA-Binding Proteins / Q000276:immunology; D003882:Dermatomyositis / Q000175:diagnosis / Q000276:immunology*; D018450:Disease Progression; D006801:Humans; D000072640:Interferon-Induced Helicase, IFIH1 / Q000276:immunology; D017563:Lung Diseases, Interstitial / Q000276:immunology; D018908:Muscle Weakness / Q000276:immunology; D009369:Neoplasms / Q000276:immunology; D009687:Nuclear Proteins / Q000276:immunology; D011379:Prognosis; D014157:Transcription Factors / Q000276:immunology; D044764:Ubiquitin-Activating Enzymes / Q000276:immunology
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D004268:DNA-Binding Proteins; D009687:Nuclear Proteins; D014157:Transcription Factors; C087950:nuclear matrix protein 2; C093517:transcriptional intermediary factor 1; C452815:IFIH1 protein, human; D000072640:Interferon-Induced Helicase, IFIH1; D044764:Ubiquitin-Activating Enzymes