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Acute rheumatic fever (ARF) is a postinfectious immune-mediated syndrome predominantly affecting children, adolescents and young adults following infection with the group A Streptococcus (Streptococcus pyogenes (Strep A). Its clinical presentation is varied, with symptoms, signs and laboratory abnormalities reflecting systemic inflammation (fever, elevated C reactive protein and erythrocyte sedimentation rate) and focal inflammation of the heart (carditis, most frequently manifest as valvulitis), joints (arthritis and arthralgia), skin (subcutaneous nodules, erythema marginatum) and brain (chorea).
Severe acute rheumatic carditis may be fatal, but mortality and morbidity due to the chronic valve lesions of rheumatic heart disease (RHD) are much greater, attributable largely to heart failure and stroke. The lesions of RHD are thought to be caused by recurrent episodes of ARF due to repeated group A streptococcal infections. In the second half of the 20th century, the global burden of ARF shifted dramatically, virtually disappearing from high-income countries to become concentrated in low- and middle-income countries and historically dispossessed and disadvantaged groups in some high-income countries, including indigenous Australians and New Zealand Māori. This epidemiological shift out of sight of wealthy populations has left ARF and RHD neglected by researchers and funders despite the persistent global burden of more than 300, 000 incident cases of ARF and more than 30 million prevalent cases of RHD.1
Prevention and control efforts for ARF and RHD are focused on primary prevention (treatment of group A streptococcal infection to prevent …
Footnotes
Contributors Both authors contributed equally.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Commissioned; internally peer reviewed.