The Significance of Autoimmunity in Myocarditis

Abstract

A growing body of evidence supports the view that some forms of human myocarditis and dilated cardiomyopathy result from a pathogenic autoimmune response. The evidence is based first on the presence of heart-specific antibodies in many patients with these diseases, including antibodies with demonstrated functional effects. These antibodies may be present before the onset of dilated cardiomyopathy and may be predictive of the course of disease in terms of deterioration of cardiac function. Depletion of the heart-specific antibodies by extracorporeal immunoadsorption may result in amelioration of disease in some patients, often continuing for long periods of time. Clinical investigations show that a subpopulation of patients with dilated cardiomyopathy benefit from immunosuppressive treatment. In one report, this subpopulation was identified as autoantibody-positive and virus-negative. Finally, animal experiments have shown that autoimmune myocarditis can be induced by viral infection and that this autoimmune response can be duplicated by immunization with a well-characterized antigen, cardiac myosin. Based on this evidence, we propose that some forms of dilated cardiomyopathy and myocarditis result from pathogenic autoimmune responses that represent the final common pathogenetic pathway of various infectious and even non-infectious injuries.