Pathogenesis of myocarditis and dilated cardiomyopathy

Adv Immunol. 2008:99:95-114. doi: 10.1016/S0065-2776(08)00604-4.

Abstract

Myocarditis is a disease with a variable clinical presentation, ranging from asymptomatic to a fatal outcome. Among the recognized causes of myocarditis are mutations in multiple genes; infection by bacterial, rickettsial, mycotic, protozoan, and viral agents; and exposure to drugs, toxins, and alcohol. Some subtypes of myocarditis, such as giant cell myocarditis or eosinophilic necrotizing myocarditis, are suspected to be caused by an autoimmune inflammation. Several lines of evidence support the involvement of autoimmunity in myocarditis. These include the production of antibodies against relevant self-antigens, the fact that myocarditis symptoms can be relieved by immunosuppressive therapy in some patients, and a co-occurrence of myocarditis with other autoimmune diseases. Most of the evidence that myocarditis is an autoimmune disease comes from animal models. In this chapter, we discuss coxsackievirus B3-induced myocarditis and myosin-induced myocarditis as models of both viral and autoimmune inflammation in the heart. The latest advances in the study of autoimmunity have been concentrated on T helper cells, particularly the newly discovered subset, Th17 cells. Experimental autoimmune myocarditis (EAM), a mouse model of myocarditis induced by cardiac myosin, is partly an IL-17-driven disease. However, we have shown recently in IL-13 knockout mice that the disease can be driven through other pathways, and that the Th1 helper cells also lead to severe heart inflammation. Most importantly, IL-17A knockout mice are not fully protected against EAM and still develop mild myocarditis. The most abundant cells in heart infiltrate in human giant cell myocarditis or EAM are monocyte/macrophages, and there is now evidence that macrophages play a decisive role in the course of EAM.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Autoantibodies / blood
  • Autoimmune Diseases / etiology
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / pathology
  • Cardiac Myosins / immunology
  • Cardiomyopathy, Dilated / etiology
  • Cardiomyopathy, Dilated / immunology*
  • Cardiomyopathy, Dilated / pathology
  • Coxsackievirus Infections / complications
  • Coxsackievirus Infections / immunology
  • Coxsackievirus Infections / virology
  • Cytokines / immunology
  • Cytokines / metabolism
  • Disease Models, Animal
  • Enterovirus B, Human / immunology
  • Humans
  • Macrophages / immunology*
  • Macrophages / metabolism
  • Monocytes / immunology*
  • Monocytes / metabolism
  • Myocarditis / etiology
  • Myocarditis / immunology*
  • Myocarditis / pathology
  • T-Lymphocytes, Helper-Inducer / immunology*
  • T-Lymphocytes, Helper-Inducer / metabolism

Substances

  • Autoantibodies
  • Cytokines
  • Cardiac Myosins