Interleukin-6-deficient mice resist development of autoimmune myocarditis associated with impaired upregulation of complement C3

Circulation. 2003 Jan 21;107(2):320-5. doi: 10.1161/01.cir.0000043802.38699.66.

Abstract

Background: Interleukin (IL)-6 regulates various aspects of the immune response. In the context of heart diseases, it has been recognized as a prognostic factor for dilated cardiomyopathy, which often results from myocarditis.

Methods and results: Using IL-6-deficient mice, we studied the role of IL-6 in a model of autoimmune myocarditis resulting from immunization with a peptide derived from cardiac alpha-myosin. Prevalence and severity of myocarditis were markedly reduced in the absence of IL-6. CD4+ T cells from immunized IL-6-deficient mice proliferated poorly on restimulation with specific antigen in vitro and did not mediate disease on adoptive transfer into IL-6-competent RAG-2-deficient mice, which otherwise lack B cells and T cells. Production of complement C3, a crucial factor for the development of myocarditis, was strongly upregulated in IL-6+/+ but not in IL-6-deficient mice after immunization.

Conclusions: Our results demonstrate that IL-6 is required for the expansion of autoimmune CD4+ T cells and the pathogenesis of autoimmune myocarditis, possibly by upregulation of complement C3.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adoptive Transfer
  • Animals
  • Autoantibodies / blood
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / pathology
  • Autoimmune Diseases / prevention & control*
  • B-Lymphocytes / immunology
  • CD4-Positive T-Lymphocytes / cytology
  • CD4-Positive T-Lymphocytes / immunology
  • Cell Division / immunology
  • Cells, Cultured
  • Complement C3 / metabolism*
  • DNA-Binding Proteins / deficiency
  • DNA-Binding Proteins / genetics
  • Disease Models, Animal
  • Disease Susceptibility / immunology
  • Immunization
  • Immunohistochemistry
  • Interleukin-6 / deficiency*
  • Interleukin-6 / genetics
  • Interleukin-6 / immunology
  • Mice
  • Mice, Inbred BALB C
  • Mice, Knockout
  • Myocarditis / complications
  • Myocarditis / immunology
  • Myocarditis / pathology
  • Myocarditis / prevention & control*
  • Myocardium / immunology
  • Myocardium / pathology
  • Peptide Fragments / immunology
  • Tumor Necrosis Factor-alpha / immunology
  • Up-Regulation
  • Ventricular Myosins / immunology

Substances

  • Autoantibodies
  • Complement C3
  • DNA-Binding Proteins
  • Interleukin-6
  • Peptide Fragments
  • Rag2 protein, mouse
  • Tumor Necrosis Factor-alpha
  • V(D)J recombination activating protein 2
  • Ventricular Myosins