ABSTRACT
At the end of the 20th century, after an apparent decline, acute rheumatic fever (ARF) now constitutes a great challenge for developed and developing countries. It is caused by a group A beta-hemolytic Streptococcus upper airways infection, but the exact pathogenetic mechanisms are not yet clear. The role of the immune system in the pathogenesis of ARF is understood better than genetic host factors. ARF can mimic many other diseases, and the diagnosis is based on clinical criteria. It is still overdiagnosed and underdiagnosed in different settings. Penicillin has greatly contributed to the reduction in the incidence and recurrence of this disease. Current schemes of prophylaxis, however, present many problems, and failures are common. Future efforts to reduce the burden of this disease should induce public health measures the vaccine strategies.