Acute rheumatic fever is caused by the body’s autoimmune response to a group A streptococcal infection, classically pharyngitis. Rheumatic heart disease refers to the long-term cardiac damage caused by either a single severe episode or multiple recurrent episodes of acute rheumatic fever. It is rheumatic heart disease that remains a significant worldwide cause of morbidity and mortality, particularly in resource-poor settings. Improved living conditions and widespread treatment of superficial group A streptococcal infections have meant acute rheumatic fever/rheumatic heart disease are now rare in developed countries although some Indigenous populations living in wealthy nations are adversely affected. Acute rheumatic fever largely affects children between the ages of 5–14 years with risk factors for acute rheumatic fever/rheumatic heart disease including age, sex, environmental influences that increase exposure to group A streptococcal infections, and host susceptibility. Since rheumatic heart disease results from cumulative damage, the peak prevalence of clinically symptomatic rheumatic heart disease occurs between the ages of 20–40 years. Conservative estimates indicate between 275,000 and 345,000 deaths occur each year from rheumatic heart disease with global burden of disease figures in 2013 calculating at least 32.9 million prevalent rheumatic heart disease cases. In 2015, rheumatic heart disease was ranked as the 43rd leading cause of years of life lost and is attributed to an annual 1.8 million years lived with disability and 9 million lost disability-adjusted life years. More than 75% of the world’s children are currently living in countries where rheumatic heart disease remains endemic. Effective rheumatic heart disease prevention, control, and management warrants prioritization if World Health Organization global targets to reduce premature deaths from cardiovascular diseases by 25% by 2025 are to be achieved.
Cumulative incidence of rheumatic fever in an endemic
region: a guide to the susceptibility of the population?