Clinical correlations with dermatomyositis-specific autoantibodies in adult Japanese patients with dermatomyositis: a multicenter cross-sectional study

Arch Dermatol. 2011 Apr;147(4):391-8. doi: 10.1001/archdermatol.2011.52.


Objective: To clarify the association of clinical and prognostic features with dermatomyositis (DM)-specific autoantibodies (Abs) in adult Japanese patients with DM.

Design: Retrospective study.

Setting: Kanazawa University Graduate School of Medical Science Department of Dermatology and collaborating medical centers. Patients A total of 376 consecutive adult Japanese patients with DM who visited our hospital or collaborating medical centers between 2003 and 2008.

Main outcome measures: Clinical and laboratory characteristics of adult Japanese patients with DM and DM-specific Abs that include Abs against Mi-2, 155/140, and CADM-140.

Results: In patients with DM, anti-Mi-2, anti-155/140, and anti-CADM-140 were detected in 9 (2%), 25 (7%), and 43 (11%), respectively. These DM-specific Abs were mutually exclusive and were detected in none of 34 patients with polymyositis, 326 with systemic sclerosis, and 97 with systemic lupus erythematosus. Anti-Mi-2 was associated with classical DM without interstitial lung disease or malignancy, whereas anti-155/140 was associated with malignancy. Patients with anti-CADM-140 frequently had clinically amyopathic DM and rapidly progressive interstitial lung disease. Cumulative survival rates were more favorable in patients with anti-Mi-2 compared with those with anti-155/140 or anti-CADM-140 (P < .01 for both comparisons). Nearly all deaths occurred within 1 year after diagnosis in patients with anti-CADM-140. Conclusion Dermatomyositis-specific Abs define clinically distinct subsets and are useful for predicting clinical outcomes in patients with DM.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Asian People
  • Autoantibodies / blood
  • Autoantibodies / drug effects
  • Autoantibodies / immunology*
  • Cross-Sectional Studies
  • Dermatomyositis / drug therapy
  • Dermatomyositis / immunology*
  • Dermatomyositis / mortality
  • Female
  • Glucocorticoids / immunology
  • Glucocorticoids / therapeutic use
  • Humans
  • Lung Diseases, Interstitial / immunology
  • Lung Diseases, Interstitial / mortality
  • Male
  • Methylprednisolone / immunology
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Prednisolone / immunology
  • Prednisolone / therapeutic use
  • Retrospective Studies
  • Treatment Outcome


  • Autoantibodies
  • Glucocorticoids
  • Prednisolone
  • Methylprednisolone