In this chapter the clinical manifestations of rheumatic fever (RF) are described. Children and adolescents living in less developed countries are primarily affected. However, a reappearance of RF is recently observed also in industrialized countries. RF is a nonsuppurative complication of sore throat by highly virulent group A streptococcus (GAS) strains with hyaluronate capsules and M protein molecules containing epitopes cross-reactive with host tissues: joints, skin, heart, and nervous system. Carditis is the most serious complication causing morbidity and mortality. Males and females are equally affected except for chorea, prevalent in girls. Host factors are important in the pathogenesis of disease and a genetic susceptibility is suggested. Its incidence is 100–200/100,000 per year in Eastern Europe, Middle East, Asia, and Australia, while in America and Western Europe, it ranges from 0.5 to 3/100,000. Diagnosis depends on clinical criteria: (1) Arthritis (80 %): migratory, painful, and responsive to aspirin. (2) Carditis (50 %): myocardium, endocardium, and pericardium are affected; endocarditis is the main manifestation with mitral regurgitation and less commonly aortic insufficiency. (3) Erythema marginatum (5 %): macular, non-pruritic rash with serpiginous erythematosus spots on the trunk and rarely on the face. (4) Subcutaneous nodules: rare, located on the extensor surface of joints. (5) Sydenham’s chorea: inflammation of the basal ganglia and caudate nucleus of the CNS, a late complication characterized by involuntary, purposeless, and uncoordinated movements of the extremities. According to the revised Jones’ criteria, the diagnosis of RF requires 2 major criteria or 1 major and 2 minor (fever, arthralgia, elevated acute phase reactants, or prolonged PR interval) with supporting evidence of antecedent GAS infection: positive throat culture or rapid test, or elevated or rising streptococcal antibody test. Accurate diagnosis and compliance to antibiotic prophylaxis are key to primary and secondary prevention of RF.
KeywordsSystemic Lupus Erythematosus Juvenile Idiopathic Arthritis Mitral Regurgitation Septic Arthritis Rheumatic Fever
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