The Role of Genes in the Pathogenesis of Rheumatic Fever and Rheumatic Heart Disease

Rheumatic heart disease is a sequel of rheumatic fever that follows an untreated group A streptococcal infection in young susceptible individuals. The disease is mediated by autoimmune reactions. Several genes related to both the innate and adaptive immune response are involved. Several HLA class II alleles have been associated with the disease. In the present review, we focus on punctual genetic polymorphisms associated with RF/RHD development, most of which are related innate immunity. The role of inflammatory cytokines as mediators of rheumatic heart lesions and a discussion of the major autoantigens recognized due to their molecular mimicry with streptococcal antigens are also presented. A vaccine against S. pyogenes is being developed, and an increase in the knowledge of the underlying mechanisms of the disease will certainly facilitate the development of an effective and safe vaccine.