Role of the immune system in the etiology of rheumatic fever

In order to summarize all the data regarding the immunological findings in RF we have elaborated the following theory about the pathogenesis of RF (fig. 1). The disease will develop only after repeated beta-hemolytic group A streptococcal throat infection in susceptible individuals. In these people, who have a specific immune response to repeated streptococcal infection due to their DR region, a decrease in suppressor activity will develop. This will lead to an increased specific humoral and cellular immune response to streptococcal antigen which cross-reacts with specific human tissue as the heart or the brain. Alternatively or concomitantly specific autoantibodies against these tissues are made. These antibodies together with the cellular response will cause the clinical manifestation seen in RF. This is only a proposed theory and still much more work has to be done to solve the enigma regarding the pathogenesis of RF.