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Acute rheumatic fever: Epidemiology and pathogenesis

Andrew Steer, MBBS, PhD, FRACP
Allan Gibofsky, MD, JD, FACP, FCLM
Section Editors
Robert Sundel, MD
Daniel J Sexton, MD
Sheldon L Kaplan, MD
Deputy Editor
Elizabeth TePas, MD, MS


The potential complications of group A Streptococcus (GAS) pharyngeal infection include both suppurative (eg, peritonsillar abscess, otitis media, sinusitis) and inflammatory, nonsuppurative conditions. Acute rheumatic fever (ARF) is one of the nonsuppurative complications (others include scarlet fever and acute glomerulonephritis [AGN]). There is a latent period of two to three weeks following the initial pharyngitis before the first signs or symptoms of ARF appear [1]. The disease presents with various manifestations that may include arthritis or arthralgia, carditis, chorea, subcutaneous nodules, and erythema marginatum.

The epidemiology and pathogenesis of ARF are reviewed here. The clinical manifestations, diagnosis, treatment, and prevention of this disorder are discussed separately. (See "Acute rheumatic fever: Clinical manifestations and diagnosis" and "Acute rheumatic fever: Treatment and prevention".)

Other complications of streptococcal tonsillopharyngitis are also discussed separately. (See "Complications of streptococcal tonsillopharyngitis".)


Rheumatic fever and rheumatic heart disease are diseases of poverty and economic disadvantage. In developing areas of the world, severe disease caused by group A Streptococcus (GAS; eg, ARF, rheumatic heart disease, glomerulonephritis, and invasive infections) is estimated to affect over 33 million people [2] and is the leading cause of cardiovascular death during the first five decades of life [3]. ARF can occur at any age, although most cases occur in children 5 to 15 years of age [4-6]. Worldwide, based upon conservative estimates, there are approximately 470,000 new cases of ARF and 275,000 deaths attributable to rheumatic heart disease each year [2,3,7,8]. Most cases occur in low- and middle-income countries and among Indigenous groups [9]. Regions with the highest rates are likely to have the least accurate data with substantial underreporting.

The mean incidence of ARF is 19 per 100,000 school-aged children worldwide [10], but it is lower (≤2 cases per 100,000 school-aged children) in the United States and other developed countries [11,12]. In many low- and middle-income countries and in certain Indigenous populations, such as those in Australia and New Zealand, the incidence of ARF is substantially higher, with some of the highest rates reported in Indigenous Australians at 153 to 380 cases per 100,000 children aged 5 to 14 years [5].

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Literature review current through: Nov 2017. | This topic last updated: Jan 03, 2017.
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