Primary episodes of acute rheumatic fever (ARF) occur mainly in children aged 5 to 14 years and are rare in people over 30 years old.[4]Carapetis JR, Steer AC, Mulholland EK, et al. The global burden of group A streptococcal diseases. Lancet Infect Dis. 2005 Nov;5(11):685-94.
http://www.ncbi.nlm.nih.gov/pubmed/16253886?tool=bestpractice.com
Recurrent episodes remain relatively common in adolescents and young adults but uncommon in those over 35 years old.[1]World Health Organization. Rheumatic fever and rheumatic heart disease: report of a WHO Expert Consultation. 2004 [internet publication].
https://apps.who.int/iris/handle/10665/42898
Overall, it is estimated that in 2019, 40.5 million people were affected by rheumatic heart disease (RHD), accounting for 10.7 million disability-adjusted life years lost and leading to 306,000 deaths.[5]Watkins DA, Johnson CO, Colquhoun SM, et al. Global, regional, and national burden of rheumatic heart disease, 1990-2015. N Engl J Med. 2017 Aug 24;377(8):713-22.
http://www.nejm.org/doi/full/10.1056/NEJMoa1603693#t=article
http://www.ncbi.nlm.nih.gov/pubmed/28834488?tool=bestpractice.com
[6]Roth GA, Mensah GA, Johnson CO, et al. Global burden of cardiovascular diseases and risk factors, 1990-2019: update from the GBD 2019 study. J Am Coll Cardiol. 2020 Dec 22;76(25):2982-3021.
https://www.sciencedirect.com/science/article/pii/S0735109720377755
http://www.ncbi.nlm.nih.gov/pubmed/33309175?tool=bestpractice.com
[Figure caption and citation for the preceding image starts]: Global prevalence and mortality rates. Source: data derived from Global Burden of Disease data 2010/2013.Zühlke, L.J., Beaton, A., Engel, M.E. et al. Group A streptococcus, acute rheumatic fever and rheumatic heart disease: epidemiology and clinical considerations. Curr Treat Options Cardio Med 19, 15 (2017). Used with permission. [Citation ends].
The greatest burden of ARF and RHD is in people in low- and middle-income countries and in populations of indigenous people living in poverty in high-income countries.[4]Carapetis JR, Steer AC, Mulholland EK, et al. The global burden of group A streptococcal diseases. Lancet Infect Dis. 2005 Nov;5(11):685-94.
http://www.ncbi.nlm.nih.gov/pubmed/16253886?tool=bestpractice.com
The highest prevalence and age-standardised mortality of RHD are in Oceania, South Asia, and central sub-Saharan Africa.[5]Watkins DA, Johnson CO, Colquhoun SM, et al. Global, regional, and national burden of rheumatic heart disease, 1990-2015. N Engl J Med. 2017 Aug 24;377(8):713-22.
http://www.nejm.org/doi/full/10.1056/NEJMoa1603693#t=article
http://www.ncbi.nlm.nih.gov/pubmed/28834488?tool=bestpractice.com
[6]Roth GA, Mensah GA, Johnson CO, et al. Global burden of cardiovascular diseases and risk factors, 1990-2019: update from the GBD 2019 study. J Am Coll Cardiol. 2020 Dec 22;76(25):2982-3021.
https://www.sciencedirect.com/science/article/pii/S0735109720377755
http://www.ncbi.nlm.nih.gov/pubmed/33309175?tool=bestpractice.com
There is no clear sex predilection for ARF, although RHD tends to be more common in females. ARF is most common in tropical countries with no seasonal variation. The highest rates of ARF have been documented in Aboriginal children in the Northern Territory of Australia and Pacific peoples, including those living in the US and New Zealand.[7]Miyake CY, Gauvreau K, Tani LY, et al. Characteristics of children discharged from hospitals in the United States in 2000 with the diagnosis of acute rheumatic fever. Pediatrics. 2007 Sep;120(3):503-8.
http://www.ncbi.nlm.nih.gov/pubmed/17766522?tool=bestpractice.com
[8]Milne RJ, Lennon DR, Stewart JM, et al. Incidence of acute rheumatic fever in New Zealand children and youth. J Paediatr Child Health. 2012 Aug;48(8):685-91.
http://www.ncbi.nlm.nih.gov/pubmed/22494483?tool=bestpractice.com
However, data from Uganda describe a higher incidence of ARF in populations with co-existing malaria, suggesting under-recognition of ARF and the value of surveillance.[9]Okello E, Ndagire E, Atala J, et al. Active case finding for rheumatic fever in an endemic country. J Am Heart Assoc. 2020 Aug 4;9(15):e016053.
https://www.ahajournals.org/doi/full/10.1161/JAHA.120.016053
http://www.ncbi.nlm.nih.gov/pubmed/32750303?tool=bestpractice.com
ARF was common in high-income countries including the US until the first half of the 20th century, when the incidence decreased because of improvements in living conditions and hygiene, which in turn led to decreased transmission of group A streptococci.[10]Quinn RW. Comprehensive review of morbidity and mortality trends for rheumatic fever, streptococcal disease, and scarlet fever: the decline of rheumatic fever. J Pediatr. 1994 Jan;124(1):9-16.
http://www.ncbi.nlm.nih.gov/pubmed/2690288?tool=bestpractice.com
In the 1980s there was a resurgence of ARF in the intermountain regions in the US, thought to be related to the emergence of virulent group A streptococci belonging to M serotypes 1, 3, and 18.[11]Veasy LG, Tani LY, Hill HR. Persistence of acute rheumatic fever in the intermountain area of the United States. J Pediatr. 1994 Jan;124(1):9-16.
http://www.ncbi.nlm.nih.gov/pubmed/7802743?tool=bestpractice.com
[12]Veasy LG, Wiedmeier SE, Orsmond GS, et al. Resurgence of acute rheumatic fever in the intermountain area of the United States. N Engl J Med. 1987 Feb 19;316(8):421-7.
http://www.ncbi.nlm.nih.gov/pubmed/3807984?tool=bestpractice.com
[13]Johnson DR, Stevens DL, Kaplan EL. Epidemiologic analysis of group A streptococcal serotypes associated with severe systemic infections, rheumatic fever, or uncomplicated pharyngitis. J Infect Dis. 1992 Aug;166(2):374-82.
http://www.ncbi.nlm.nih.gov/pubmed/1634809?tool=bestpractice.com
In addition, changing patterns of antibiotic use, in particular the movement away from using antibiotics for treating group A streptococcal pharyngitis in countries with low rates of ARF, may also have affected the epidemiology of group A streptococcal diseases. However, overall the incidence of ARF in the US is unknown, as it is no longer a nationally notifiable disease.[14]Centers for Disease Control and Prevention. Group A streptococcal disease. Acute rheumatic fever. Jun 2022 [internet publication].
https://www.cdc.gov/groupastrep/diseases-hcp/acute-rheumatic-fever.html