Background: There is no systematic overview of prospective studies of incidence of acute rheumatic fever (ARF) in the world.
Aim: To summarise all population-based studies of the incidence of ARF world wide.
Method: A systematic review of prospective population-based studies of the overall mean and annual specific incidence of the first episode of ARF was carried out.
Results: A systematic literature search identified 10 eligible studies from 10 countries on all continents, except Africa. The overall mean incidence rate of first attack of ARF was 5–51/100 000 population (mean 19/100 000; 95% CI 9 to 30/100 000). A low incidence rate of ⩽10/100 000 per year was found in America and Western Europe, while a higher incidence (>10/100 000) was documented in Eastern Europe, Middle East (highest), Asia and Australasia. Studies with longitudinal data displayed a falling incidence rate over time.
Conclusion: Despite an apparent fall in incidence over time, ARF incidence rates remain relatively high in non-Western countries. No information is available for Africa.
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Funding: KBT received financial support from the Groote Schuur Hospital Facility Board during the conduct of this work. BMM is funded in part by the Medical Research Council of South Africa, the National Research Foundation of South Africa and the Wellcome Trust (UK)
Competing interests: None.