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Acute rheumatic fever (ARF) is a multisystem disease resulting from an autoimmune reaction to infection with group A streptococcus. Although many parts of the body may be affected, almost all of the manifestations resolve completely. The exception is cardiac valvular damage [rheumatic heart disease (RHD)], which may persist after the other features have disappeared.

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ARF and RHD are diseases of poverty. They were common in all countries until the early twentieth century, when their incidence began to decline in industrialized nations. This decline was largely attributable to improved living conditions—particularly less crowded housing and better hygiene—which resulted in reduced transmission of group A streptococci. The introduction of antibiotics and improved systems of medical care had a supplemental effect. Recurrent outbreaks of ARF began in the 1980s in the Rocky Mountain states of the United States, where elevated rates persist.

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The virtual disappearance of ARF and reduction in the incidence of RHD in industrialized countries during the twentieth century unfortunately was not replicated in developing countries, where these diseases continue unabated. RHD is the most common cause of heart disease in children in developing countries and is a major cause of mortality and morbidity in adults as well. It has been estimated that between 15 and 19 million people worldwide are affected by RHD, with approximately one-quarter of a million deaths occurring each year. Some 95% of ARF cases and RHD deaths now occur in developing countries.

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Although ARF and RHD are relatively common in all developing countries, they occur at particularly elevated rates in certain regions. These “hot spots” are sub-Saharan Africa, Pacific nations, Australasia, and the Indian subcontinent (Fig. 322-1). Unfortunately, most developing countries do not currently have coordinated, register-based RHD control programs, which are proven to be cost-effective in reducing the burden of RHD. Enhancing awareness of RHD and mobilizing resources for its control in developing countries is an issue requiring international attention.

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Figure 322-1
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Prevalence of rheumatic heart disease in children aged 5–14 years. Circles within Australia and New Zealand represent indigenous populations, and also Pacific Islanders in New Zealand. (From JR Carapetis et al: Lancet Infect Dis. Copyright 2005, with permission from Elsevier.)

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ARF is mainly a disease of children aged 5–14 years. Initial episodes become less common in older adolescents and young adults and are rare in persons aged >30 years. By contrast, recurrent episodes of ARF remain relatively common in adolescents and young adults. This pattern contrasts with the prevalence of RHD, which peaks between 25 and 40 years. There is no clear gender association for ARF, but RHD more commonly affects females, sometimes up to twice as frequently as males.

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Organism Factors

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Based on currently available evidence, ARF is exclusively caused by infection of the upper respiratory tract with group A ...

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