The genetics of rheumatic fever: relationship to streptococcal infection and autoimmune disease.

A large body of evidence suggests that patients with rheumatic fever immunologically respond abnormally both at a humoral and cellular level to streptococcal antigens cross-reactive with mammalian tissues. Implicit in this concept is that this abnormal immune response is genetically programmed. Using a monoclonal antibody called D8/17, a B cell marker has now been identified in 90-100% of all patients with rheumatic fever tested in 5 different geographical and ethnic populations. This trait appears to be inherited in an autosomal recessive fashion. This recognition of a B cell marker unique to individuals with rheumatic fever has important public health implications with respect to identification of individuals susceptible to rheumatic fever, possible prevention of disease and the recognition of prime candidates for future streptococcal vaccines.