Definition and epidemiology
Acute rheumatic fever (ARF) is a delayed, immune-mediated multisystem inflammatory sequela of group A streptococcal (GAS) pharyngitis, arising roughly 2–4 weeks after the throat infection. It affects principally children aged 5–15 years and is the precursor to rheumatic heart disease (RHD), the leading cause of acquired paediatric heart disease worldwide. ARF and RHD remain major causes of cardiovascular morbidity in India and other low- and middle-income settings, where they are classed as moderate- to high-risk populations under the revised criteria.
Clinical features
ARF is a clinical syndrome; the cardinal features are the major Jones criteria:
- Carditis — clinical and/or subclinical (echocardiographic valvulitis, most often mitral regurgitation); the most serious manifestation and the only one that causes lasting damage.
- Arthritis — classically a migratory polyarthritis of large joints; in moderate/high-risk populations monoarthritis and polyarthralgia also count as major.
- Sydenham chorea — may appear in isolation and late.
- Erythema marginatum — evanescent, non-pruritic, trunk and proximal limbs.
- Subcutaneous nodules — firm, painless, over extensor surfaces; associated with carditis.
Minor criteria include fever, raised acute-phase reactants (ESR, CRP), arthralgia, and a prolonged PR interval — with risk-stratified thresholds: fever ≥38.0°C (moderate/high-risk) vs ≥38.5°C (low-risk); ESR ≥30 mm/h (moderate/high-risk) vs ≥60 mm/h (low-risk); and CRP ≥3.0 mg/dL in both risk groups.
Diagnosis
A diagnosis of an initial attack requires evidence of antecedent GAS infection (positive throat culture/RADT or raised/rising antistreptococcal antibody titre) plus 2 major, or 1 major and 2 minor, criteria. The 2015 AHA revision made two key changes: it incorporated Doppler echocardiography to detect subclinical carditis as a major criterion, and it separated low- versus moderate/high-risk populations, loosening arthritis and minor-criterion thresholds where ARF is endemic. Recurrent attacks may be diagnosed with 2 major, 1 major + 2 minor, or 3 minor criteria in a patient with established RHD.
See the full criteria: Rheumatic Fever (Jones Criteria)
Red flags
- Carditis with heart failure — pulmonary oedema, gallop, new significant murmur; needs urgent admission.
- New high-grade or complete AV block.
- Chorea that is severe or disabling.
- Any confirmed ARF establishes lifelong relapse risk — each recurrence worsens valvular damage, so prophylaxis adherence is itself a safety issue.
Management overview
Management has three strands. (1) Eradicate GAS with a penicillin-class antibiotic (a macrolide-class agent if penicillin-allergic). (2) Suppress inflammation — an NSAID-class agent (salicylate or naproxen) for arthritis; corticosteroid-class agents are used for severe carditis with heart failure at clinician discretion. (3) Secondary prophylaxis — long-term intramuscular benzathine penicillin (penicillin-class) is the cornerstone to prevent recurrent GAS and progressive RHD, continued for years and stratified by carditis/RHD status. Chorea is usually self-limiting; symptomatic neuromodulating agents are reserved for disabling cases. Echocardiography at diagnosis and on follow-up guides prognosis and prophylaxis duration. Drug classes only are named here; dosing is outside the scope of this reference.
References
- Gewitz MH, et al. Circulation. 2015;131(20):1806–1818 (AHA revised Jones criteria).
- WHO technical guidance on rheumatic fever and rheumatic heart disease.
- Nelson Textbook of Pediatrics, 21st ed.
Decision support for qualified clinicians only — verify against current primary guidelines and your clinical judgement.