Rheumatic fever in pediatric patients in 1997.

Nine cases of rheumatic fever were seen from 1982 to 1996. The diagnosis wa s based on Jones criteria. Four of eight children had carditis characterize d by mitral regurgitation with or without aortic regurgitation and/or atrio ventricular conduction disturbances. The outcome was favorable in all the p atients who had carditis initially; one of the patients without initial car ditis developed permanent cardiac lesions during a recurrence with carditis . In industrialized countries, the incidence of rheumatic fever declined st arting early in the XXth century, then dropped sharply after World War II, and is now extraordinarily low (mean annual incidence, 0.5/100000 schoolage children). In developing countries, by contrast, rheumatic fever was recog nized only after World War II and remains endemic (mean annual incidence, 1 00 to 200/100000 schoolage children), contributing a substantial proportion of cases of cardiovascular disease. The diagnosis is difficult and rests o n clinical grounds since there is no specific laboratory test. Diagnostic d elays are potentially serious. Acute attacks should be managed as therapeut ic emergencies. Prevention of recurrences rests on long-term antimicrobial therapy. Rheumatic fever is a disease process resulting from an inappropria te immune response to pharyngitis due to a beta-hemolytic group A streptoto ccus (BHAS). A low standard of living may be a factor in developing countri es but fails to explain the epidemic flares seen in these areas or the resi dual background incidence in industrialized countries. A role of host-relat ed susceptibility to the disease has not been demonstrated. The type-specif ic surface M protein, the main factor associated with high virulence, carri es a specific epitope on its distal portion. Rheumatogenic strains have bee n identified; most produce mucoid colonies. At a given point in time, withi n a given serotype, the virulence of a specific strain increases. Temporal and spatial variations of observed types contribute additional complexity. Adhesion of the organisms is followed by release of streptococcal degradati on products that share antigenic determinants with human tissues including the heart, the synovium, and the neurons. The hyaluronate capsule and M pro tein of the organisms are capable of initiating immune responses, their pre sentation to CD4+ T-cells results in lymphokine production, an acute phase humoral response, and a cell-mediated response potentially responsible for permanent valvular damage. In France, the standard of care is to prescribe antimicrobial therapy to all patients with pharyngitis or tonsillitis witho ut performing tests to identify the causative agent. The introduction of te sts for the rapid recognition in routine clinical practice of BHAS, which a ccount for only 20 to 30% of all cases of pharyngitis and tonsillitis, shou ld allow a more rational approach to the treatment of these infections. Res erving antimicrobial therapy to those patients with BHAS should not result in an increase in the incidence of rheumatic fever.