Rheumatic fever is a prominent cause of serious valvular heart disease. Acute rheumatic fever and subsequent rheumatic heart disease remain important cardiovascular problems in the tropical and subtropical developing countries of South America, Africa, the Middle East, and Asia, and there have been outbreaks in the United States in recent years.37 Although preventable, rheumatic fever occurs more frequently because of overcrowding, the deceptive self-limited nature of symptoms in streptococcal pharyngitis, and the mild and often clinically inapparent nature of streptococcal infections. The availability of penicillin to treat these infections, living conditions that are less crowded than formerly, and evolution of different strains of Streptococcus have made rheumatic fever uncommon in the United States, although the incidence remains high in subgroups such as blacks, Puerto Ricans, Mexican Americans, and Native Americans (see Chap. 62). Because this disease has not been eradicated in this country, there is a need to define its incidence and prevalence more accurately as well as those of the infective endocarditis that may follow in order to pinpoint those at risk (see also Chap. 82).
An estimated 1.8 million persons have rheumatic heart disease in the United States, more than 6 per 1000 persons.2 About 15 percent of these persons are limited in activity because of the resulting chronic carditis.6 There is no national estimate of annual incidence. A study in Tennessee reported a range from 0.5 to 1.88 new cases per 100,000 school-aged children in 19771981, with the lowest rates in the affluent suburbs.37 Occurrence tends to be concentrated in the lower socioeconomic subgroups, perhaps due to factors of nutrition, hygiene, and access to medical care. Rheumatic fever is rare before age 3, occurring most frequently between 5 and 15 years of age, when streptococcal infections are most frequent. During epidemics of streptococcal pharyngitis, the rheumatic fever attack rate may be 3 percent, whereas in endemic situations it is usually only 0.3 percent (see also Chap. 62).
With the decline in rheumatic fever in the United States, its clinical manifestations also have moderated so that carditis is detected in fewer than 20 percent of acutely affected patients.38 The annual mortality has declined to about 5000 deaths per year. Because the cardiac sequelae of rheumatic fever are still seen in adults and adequate treatment can reduce attacks by 90 percent, rheumatic fever and rheumatic heart disease remain the two most preventable serious cardiovascular disorders. It seems clear that at least part of the decline in rheumatic fever was due to prompt antistreptococcal treatment by physicians. The decline in rheumatic fever, however, appears to have antedated the advent of antistreptococcal agents. We are currently unable to explain the reasons for the decline in rheumatic fever definitely, possibly because we do not fully understand its etiologic factors (see Chap. 62).
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