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 <or=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.