Rheumatic fever

Rheumatic fever is an autoimmune inflammatory disorder triggered by an aberrant immune response to a preceding Group A Streptococcus infection, most commonly pharyngitis or occasionally impetigo. The pathogenesis involves molecular mimicry where antibodies directed against the bacterial pathogen cross-react with host tissues. Clinically, it manifests as a systemic illness that frequently progresses to chronic valvular pathology known as rheumatic heart disease. ^[1][3][4]

Clinical presentation typically includes fever, migratory polyarthritis, chorea, and occasionally erythema marginatum, with cardiac involvement occurring in approximately half of cases. Diagnostic confirmation relies on established clinical criteria, such as the revised Jones Criteria, supplemented by evidence of a recent streptococcal infection. Cardiac sequelae, primarily valvular damage, may develop after a single severe episode or accumulate following recurrent attacks, potentially leading to arrhythmias or heart failure. Current management focuses on symptomatic relief rather than altering the underlying disease trajectory. ^[1][2][3][4]

Globally, rheumatic fever imposes a substantial disease burden, with hundreds of thousands of annual deaths concentrated in low-resource settings and millions living with associated valvular damage. Geographic and demographic distributions reveal marked disparities, with the highest prevalence observed among Indigenous Australian, Māori, and Pacific Islander communities, as well as populations facing socioeconomic disadvantage. In contrast, incidence has declined sharply in high-income nations since the mid-twentieth century, though sporadic community outbreaks continue to emerge in developed regions. ^[1][2][4]

The condition does not transmit directly between individuals; rather, it arises as a post-infectious complication following human-to-human spread of Group A Streptococcus via respiratory droplets or direct contact during pharyngitis or cutaneous infections. Susceptibility to developing the autoimmune sequela depends on host genetic factors and prior exposure to the pathogen. ^[1]

Primary prevention centers on the prompt identification and antibiotic treatment of streptococcal pharyngitis, alongside broader public health initiatives targeting overcrowding, domestic air pollution, and poverty. Secondary prevention utilizes prolonged antibiotic prophylaxis to prevent recurrence and subsequent cardiac deterioration in previously affected individuals. Emerging strategies emphasize equitable healthcare delivery, enhanced screening protocols utilizing echocardiography, and ongoing research into novel group A streptococcal vaccines to reduce long-term disease burden. ^[2][3][4]

1. 1Wikipedia contributors. Rheumatic fever - Wikipedia [Internet]. Wikipedia. cited 4 Sept 2026.Available from: https://en.wikipedia.org/wiki/Rheumatic_fever
2. 2Rwebembera J et al. Acute rheumatic fever and rheumatic heart disease: updates in diagnosis and treatment. Curr Opin Pediatr. 2024 Oct 1. PMID: 39254753. doi: 10.1097/MOP.0000000000001384.PubMed: https://pubmed.ncbi.nlm.nih.gov/39254753/
3. 3Carapetis 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/
4. 4Hirani 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/