Rheumatic Fever
Basics
Description
- Acute rheumatic fever (ARF) is an autoimmune, inflammatory response to infection with group A Streptococcus (GAS) that affects multiple organ systems.
- Untreated acute disease can lead to chronic rheumatic heart disease (RHD).
- Recurrence is common without adequate antibiotic treatment.
Pediatric Considerations
Most cases occur in children aged 5 to 15 years; rare in children aged <5 years
Epidemiology
- ARF and RHD are largely restricted to low-income countries and marginalized sections of wealthy countries.
- Male = female; females more likely to develop chorea and RHD.
- Endemic regions include South Pacific, indigenous populations of Australia and New Zealand, Africa, and Asia (1).
Incidence
- Worldwide, incidence has been declining for 25 years. The large majority of new cases are in developing countries (1).
- Mean worldwide incidence ranges from 8 to 51/100,000 school-aged children; in endemic regions, prevalence can be >1,000 cases per 100,000 people (1).
- Incidence of ARF in the United States is currently <3.4 to 2/100,000 school-aged children (1).
Prevalence
- In developing areas, RHD affects >33 million people and is the leading cause of cardiovascular death during the first 5 decades of life.
- Prevalence has been rising due to improved medical care and longer survival.
Etiology and Pathophysiology
- ARF most commonly occurs 2 to 3 weeks after GAS pharyngitis infection, but GAS impetigo may also be a proceeding infection.
- Although pathogenicity is not completely understood, expert consensus implicates genetic and molecular mimicry leading to an inflammatory cascade as key to disease development.
- Immune cross reactive response contributes to joint involvement due to accumulation of immune complexes.
Genetics
- Susceptibility is associated with certain indigenous populations.
- ARF is heritable, polygenic, and displays variable and incomplete penetrance.
Risk Factors
Poverty, household crowding, genetic susceptibility, ethnic predisposition, and social disadvantage are the strongest risk factors.
General Prevention
- Primary prevention: Appropriate treatment of streptococcal infection prevents ARF in most cases.
- Secondary prevention: long-term antibiotic prophylaxis (up to 5 to 10 years) to prevent recurrence (1)
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Citation
Domino, Frank J., et al., editors. "Rheumatic Fever." 5-Minute Clinical Consult, 33rd ed., Wolters Kluwer, 2025. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688813/all/Rheumatic_Fever.
Rheumatic Fever. In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2025. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688813/all/Rheumatic_Fever. Accessed November 3, 2024.
Rheumatic Fever. (2025). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (33rd ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688813/all/Rheumatic_Fever
Rheumatic Fever [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2025. [cited 2024 November 03]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688813/all/Rheumatic_Fever.
* Article titles in AMA citation format should be in sentence-case
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