Pathogenesis of Acute Rheumatic Fever

Abstract Acute rheumatic fever is a multiorgan inflammatory disorder affecting the heart, joints, brain, and skin. The pathogenic mechanism of molecular mimicry involves autoantibodies and T cells directed against group A streptococcal cell wall components and heart or brain antigens that have been extensively investigated. Autoantibodies and T-cell responses in rheumatic heart disease and Sydenham chorea induce valve disease with heart murmurs and movement and behavioral disturbances, respectively. In carditis, autoantibodies and T cells target valve proteins while in Sydenham chorea the autoantibodies deposit in the brain and target the basal ganglia and dopaminergic neurons.