Myositis in mixed connective tissue disease: a unique syndrome characterized by immunohistopathologic elements of both polymyositis and dermatomyositis

Arq Neuropsiquiatr. 2004 Dec;62(4):923-34. doi: 10.1590/s0004-282x2004000600001. Epub 2004 Dec 15.

Abstract

Objective: To characterize the inflammatory cells, the expression pattern of adhesion molecules (ICAM-1 and VCAM-1), membrane attack complex (C5b-9), and major histocompatibility complex (MHC) antigens in muscle biopsy of mixed connective tissue disease (MCTD).

Method: We studied 14 patients with MCTD, and compared to 8 polimyositis (PM) patients, 5 dermatomyositis (DM) and 4 dystrophies. Inflammatory cells were examined for CD4+, CD8+, memory and naive T cells, natural killer cells, and macrophages. Expression of MHC-I and -II, ICAM-1, VCAM-1 and C5b -9 were characterized on muscle fibers and vessels.

Results: Morphological analysis displayed a pattern of PM. Immunohistochemical study revealed a decreased number of capillaries, predominance of CD4+ and B cells in perivascular regions and predominance of CD8+ and CD45RO+ in endomysial regions. The expression of MHC-I on vessels and on degenerated muscle fibers, MHC-II expression on vessels and perifascicular muscle fibers, and the expression of ICAM-1 / VCAM-1 on endothelial cells indicated both vascular and cellular-immune mediated processes causing the muscular lesion.

Conclusion: Our findings revealed a mixed mechanism in MCTD, both vascular involvement as DM, and cell-mediated like PM.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Age Distribution
  • Antigens, CD / immunology
  • Autoantibodies / blood
  • CD4-CD8 Ratio
  • Complement Membrane Attack Complex / immunology
  • Dermatomyositis* / immunology
  • Dermatomyositis* / pathology
  • Female
  • Gene Expression Regulation / immunology
  • Humans
  • Intercellular Adhesion Molecule-1 / immunology*
  • Major Histocompatibility Complex / immunology*
  • Male
  • Middle Aged
  • Mixed Connective Tissue Disease* / immunology
  • Mixed Connective Tissue Disease* / pathology
  • Prejudice
  • Prospective Studies
  • Vascular Cell Adhesion Molecule-1 / immunology*

Substances

  • Antigens, CD
  • Autoantibodies
  • Complement Membrane Attack Complex
  • Vascular Cell Adhesion Molecule-1
  • Intercellular Adhesion Molecule-1