Genetic and immunological differences between Japanese patients with diffuse scleroderma and limited scleroderma

J Rheumatol. 1994 Jan;21(1):111-4.

Abstract

Objective: To study the association between HLA-DR and scleroderma (SSc), subsets of SSc, and autoantibodies in SSc.

Methods: HLA-DR antigens were determined in 45 Japanese patients with SSc. The association between HLA-DR and SSc, subsets of SSc, and autoantibodies was analyzed in 22 patients with SSc excluding mixed connective tissue disease (MCTD)/overlap syndrome (OL).

Results: When the 20 patients with MCTD and 3 patients with OL were excluded from the original patient group, a significant increase of HLA-DR2 was observed (59 vs 29% of controls, p < 0.01). The frequency of DR2 increased to 69% in patients with diffuse SSc (p < 0.01). DR1, which was not found in diffuse SSc, was found in 2 of 9 patients with limited SSc. The frequency of DR2 was significantly higher in patients with antitopoisomerase I (10/12, 83%, p < 0.05). In contrast, DR1 was found only in 2 patients with anticentromere antibodies (ACA), and all 5 patients with ACA had no HLA-DR2 (p < 0.01).

Conclusion: Our results suggest that different HLA-DR markers may be associated with the production of distinct autoantibodies in diffuse SSc and limited SSc.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antibodies / analysis
  • Antibody Specificity
  • Autoantibodies / immunology
  • Centromere / immunology
  • DNA Topoisomerases, Type I / immunology
  • HLA-DR Antigens / analysis
  • Humans
  • Scleroderma, Localized / genetics*
  • Scleroderma, Localized / immunology*
  • Scleroderma, Localized / physiopathology
  • Scleroderma, Systemic / genetics*
  • Scleroderma, Systemic / immunology*
  • Scleroderma, Systemic / physiopathology

Substances

  • Antibodies
  • Autoantibodies
  • HLA-DR Antigens
  • DNA Topoisomerases, Type I