• C Olivier.
• Service de Pédiatrie Générale, Hôpital Louis Mourier, Colombes, France.
• Pathol. Biol. 1998 Dec 1; 46 (10): 802-12.

AbstractNine cases of rheumatic fever were seen from 1982 to 1996. The diagnosis was based on Jones criteria. Four of eight children had carditis characterized by mitral regurgitation with or without aortic regurgitation and/or atrioventricular conduction disturbances. The outcome was favorable in all the patients who had carditis initially; one of the patients without initial carditis developed permanent cardiac lesions during a recurrence with carditis. In industrialized countries, the incidence of rheumatic fever declined starting early in the XXth century, then dropped sharply after World War II, and is now extraordinarily low (mean annual incidence, 0.5/100,000 schoolage children). In developing countries, by contrast, rheumatic fever was recognized only after World War II and remains endemic (mean annual incidence, 100 to 200/100,000 schoolage children), contributing a substantial proportion of cases of cardiovascular disease. The diagnosis is difficult and rests on clinical grounds since there is no specific laboratory test. Diagnostic delays are potentially serious. Acute attacks should be managed as therapeutic emergencies. Prevention of recurrences rests on long-term antimicrobial therapy. Rheumatic fever is a disease process resulting from an inappropriate immune response to pharyngitis due to a beta-hemolytic group A streptotoccus (BHAS). A low standard of living may be a factor in developing countries but fails to explain the epidemic flares seen in these areas or the residual background incidence in industrialized countries. A role of host-related susceptibility to the disease has not been demonstrated. The type-specific surface M protein, the main factor associated with high virulence, carries a specific epitope on its distal portion. Rheumatogenic strains have been identified; most produce mucoid colonies. At a given point in time, within 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 degradation products that share antigenic determinants with human tissues including the heart, the synovium, and the neurons. The hyaluronate capsule and M protein of the organisms are capable of initiating immune responses; their presentation 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 without performing tests to identify the causative agent. The introduction of tests for the rapid recognition in routine clinical practice of BHAS, which account for only 20 to 30% of all cases of pharyngitis and tonsillitis, should allow a more rational approach to the treatment of these infections. Reserving antimicrobial therapy to those patients with BHAS should not result in an increase in the incidence or rheumatic fever.

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