Role of autoimmunity in rheumatic fever

摘要

Rheumatic fever is the prototype of postinfectious autoimmune disease and is caused by untreated Streptococcus pyogenes throat infection in susceptible children. This review presents the major immune-mediated events leading to carditis, valvulitis and Sydenham’s chorea (SC). The role of genetic factors in susceptibility is also discussed. In the last 10–15 years, the pathogenesis of rheumatic autoimmune reactions has been clarified. A brief review on humoral immune response, focusing on crossreactive antibodies against laminin, vimentin and cardiac myosin, and the recent data on specific crossreactive antibodies against streptococcal antigens and lysogangliosides and tubulin from neuronal cells involved with the autoimmune reactions in SC is presented. T lymphocytes are the major effectors of crossreactivity between streptococcal and human proteins leading to rheumatic heart disease. Heart tissue T-cell infiltration is facilitated by adhesion molecules, myosin and laminin antibodies. A cytokine balance favoring T-helper 1 inflammatory cytokines and few IL-4-producing cells in heart valves leads to the progression and maintenance of valvular lesions.