In spite of a limited but very disturbing resurgence of rheumatic fever in the late 1980s, rheumatic fever is not considered a major problem in the United States. Rheumatic fever and its clinically significant sequela, rheumatic heart disease, are a major health problem in nonindustrial countries occupied by two-thirds of the world population, where it is estimated that 10 to 20 million new cases of rheumatic fever occur yearly. 1 It is distressing that a potentially preventable disease remains, worldwide, the chief cause of mortality from heart disease during the first 50 years of life. 2

Decades of intensive effort by dedicated investigators from several disciplines including infectious diseases, immunology, pathology, cardiology, rheumatology, neurology and genetics have failed to define the precise pathogenesis of rheumatic fever and rheumatic heart disease. There is a strong consensus, however, that this perplexing disease represents an autoimmune response in genetically predisposed individuals to an untreated group A streptococcal pharyngitis. 3-6