Rheumatic fever

Rheumatic fever, including acute rheumatic fever (ARF), is described as an autoimmune disorder triggered by group A Streptococcus infection ^[1][2]. The provided sources characterize ARF as following pharyngitis and, in one source, impetigo, and note that the longer-term cardiac sequelae are termed rheumatic heart disease (RHD) ^[1][2]. The disease is indexed in the supplied metadata as ICD-10 I00 and ICD-11 BA40 [disease metadata].

The sources emphasize a disease spectrum in which ARF may progress to RHD with persistent cardiac valve damage ^[2]. Long-term cardiac valve injury may result from a single severe episode or from multiple recurrent episodes of illness ^[1]. RHD is described as a major source of morbidity and mortality, especially when valve damage becomes chronic ^[1][2]. The provided material does not supply a detailed symptom list or organ-system chronology beyond the cardiac sequelae and the use of clinical criteria for diagnosis ^[1][2].

The burden of rheumatic fever and its sequelae is described as global, with particular impact in resource-poor settings and among socioeconomically disadvantaged populations ^[1][2]. One source notes that RHD is a notable cause of morbidity and mortality around the world, while another specifies that the greatest burden falls on populations affected by poverty and broader social determinants of health ^[1][2]. The material also notes an interplay of social determinants and genetic factors in overall risk, but does not provide population rates, regional incidence figures, or outbreak data ^[2].

The disease is not described as directly transmitted person to person in the supplied sources. Instead, rheumatic fever follows antecedent group A Streptococcus infection, specifically pharyngitis and, in one source, impetigo ^[1][2]. The relevant exposure mechanism in the provided material is therefore prior streptococcal infection rather than a separate transmission pathway for rheumatic fever itself ^[1][2].

The supplied sources identify children and adolescents as the population in which ARF is described, and they repeatedly emphasize higher burden among socioeconomically disadvantaged populations and people living in poverty ^[2][1]. One source also notes an interplay of social determinants of health and genetic factors in determining overall risk ^[2]. No additional source-backed high-risk clinical subgroups are specified in the provided material.

The provided sources indicate that prevention efforts focus on primordial, primary, and secondary prevention, including advances in group A Streptococcus vaccine development, improved antibiotic formulations for secondary prevention, and decentralized programmatic implementation to strengthen health-care delivery ^[2]. Another source notes progress in echocardiographic screening for early detection of rheumatic heart disease ^[1]. Specific vaccine schedules, prophylaxis regimens, or exposure-control measures are not detailed in the supplied material ^[1][2].

For surveillance purposes, rheumatic fever should be interpreted as a post-streptococcal autoimmune syndrome with important downstream cardiac consequences rather than as an isolated acute infection ^[1][2]. The sources state that diagnosis remains reliant on clinical features using the Jones Criteria, with newer molecular and echocardiographic approaches mentioned as evolving tools ^[1][2]. In monitoring contexts, cases should therefore be considered alongside evidence of antecedent group A Streptococcus infection and, when available, evidence of valve involvement or progression to rheumatic heart disease ^[1][2].

1. 1 Carapetis JR et al. Acute rheumatic fever and rheumatic heart disease. Nat Rev Dis Primers. 2016 Jan 14. PMID: 27188830. doi: 10.1038/nrdp.2015.84. PubMed: https://pubmed.ncbi.nlm.nih.gov/27188830/
2. 2 Hirani K et al. Acute rheumatic fever. Lancet. 2025 Jun 14. PMID: 40484016. doi: 10.1016/S0140-6736(25)00185-0. PubMed: https://pubmed.ncbi.nlm.nih.gov/40484016/
3. 3 Baddour LM et al. Infective Endocarditis in Adults: Diagnosis, Antimicrobial Therapy, and Management of Complications: A Scientific Statement for Healthcare Professionals From the American Heart Association. Circulation. 2015 Oct 13. PMID: 26373316. doi: 10.1161/CIR.0000000000000296. PubMed: https://pubmed.ncbi.nlm.nih.gov/26373316/
4. 4 Rheumatic fever. Clinical Cardiology. 2013. doi: 10.1093/med/9780199685288.003.1838_update_001. DOI: https://doi.org/10.1093/med/9780199685288.003.1838_update_001
5. 5 Rheumatic Fever. The Lancet. 1909. doi: 10.1016/s0140-6736(00)44611-8. DOI: https://doi.org/10.1016/s0140-6736(00)44611-8
6. 6 Rheumatic Fever. Clinical Diagnosis—Cardiovascular System. 2004. doi: 10.5005/jp/books/10125_34. DOI: https://doi.org/10.5005/jp/books/10125_34

ICD-10

I00

ICD-11

BA40

Total cases

2K

Peak month

2025-12

Coverage

1 reporting countries · 2016-01-01 → 2026-05-01