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- Acute rheumatic fever (ARF) is a delayed inflammatory sequela of group A Streptococcus (GAS) tonsillopharyngitis that affects multiple organ systems.
- Can lead to rheumatic heart disease (RHD)
- Recurrence in adults and children is common if antibiotic prophylaxis is withheld.
- Systems affected: cardiovascular, nervous, hematologic/lymphatic/immunologic, skin/exocrine, musculoskeletal
Can affect any age but most common ages 5 to 15 years
- ARF and RHD are now largely restricted to developing countries and some poor populations of wealthy countries.
- Largely disease of poverty and economic disadvantage
- Male = female, but females more likely to develop chorea
- Can occur as an epidemic
- Worldwide, incidence (new cases) has been declining for decades, attributed to increasing antibiotic use and improved living conditions. Most new cases are in developing countries.
- In early studies, ARF developed in 3% of children with untreated GAS pharyngitis.
- Incidence of ARF in the United States in the 1960s was 13.3/100,000 and is currently <1/100,000.
- Mean incidence of ARF is 19/100,000 school aged children worldwide.
- 95% of cases currently occur in developing countries.
Worldwide, >15 million people have RHD, and prevalence has been rising due to improved medical care and longer survival (despite decreasing incidence of ARF).
Etiology and Pathophysiology
- Preceded by tonsillopharyngitis of GAS, also known as Streptococcus pyogenes, a gram–positive organism
- Molecular mimicry: Antibodies against M protein on GAS cross-reacts with cardiac and vessel endothelial proteins, leading to an inflammatory cascade.
- Susceptibility is associated with certain genetic polymorphisms, including toll-like receptors, cytokines, and human leukocyte antigen genes; not fully understood
- ARF appears to be a heritable disease and susceptibility is most likely polygenic.
- Increased susceptibility in certain populations, including Australian aborigines, New Zealand Maori, and Pacific Islanders
Genetic susceptibility and possible increased risk with iron deficiency or low serum albumin
- Primary prevention: Antibiotics are effective at reducing incidence of ARF after known or suspected GAS pharyngitis. Number needed to treat is 100 (1). Appropriate treatment of streptococcal pharyngitis prevents ARF in most cases.
- Secondary prevention: long-term antibiotic prophylaxis to prevent recurrence which can lead to RHD