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This is a clinician-written, evidence-based summary aligned to the 2026 MLA Content Map. It is intended for medical students and junior doctors preparing for the UKMLA. Always cross-reference with NICE guidance, local protocols, and clinical judgement.
Key points
- Autoimmune reaction 2–4 weeks after Group A Streptococcal (GAS) pharyngitis — molecular mimicry between streptococcal M protein and cardiac tissue
- Revised Jones criteria: 2 major or 1 major + 2 minor criteria + evidence of preceding GAS infection
- Major criteria: carditis, polyarthritis (migratory, large joints), Sydenham chorea, erythema marginatum, subcutaneous nodules
- Minor criteria: fever, arthralgia, raised ESR/CRP, prolonged PR interval
- Treatment: bed rest, aspirin/NSAIDs for arthritis, corticosteroids for severe carditis, penicillin V (or benzathine penicillin IM) prophylaxis to prevent recurrence
Overview
Rheumatic fever (RF) is an acute inflammatory disease that occurs as a delayed autoimmune response to Group A Streptococcal pharyngitis. The pathogenesis involves molecular mimicry — antibodies directed against streptococcal M protein cross-react with cardiac, joint, skin, and CNS antigens. Carditis is the most serious manifestation, as it can cause permanent valvular damage (rheumatic heart disease), most commonly affecting the mitral valve (mitral stenosis being the classic late sequela). RF typically affects children aged 5–15 years. While now rare in the UK, it remains a major cause of acquired heart disease in developing countries.
Epidemiology
RF is now very rare in the UK and developed countries due to improved living conditions and access to antibiotics for streptococcal pharyngitis. However, it remains a major global health problem, causing approximately 300,000 deaths annually worldwide. Highest prevalence in sub-Saharan Africa, South Asia, and Indigenous Australian and New Zealand populations. Peak incidence age 5–15 years. Recurrence rate is high without prophylaxis — each episode increases the risk of permanent cardiac damage.
Clinical Features
Symptoms
History of sore throat 2–4 weeks earlier (often forgotten)
Fever
Migratory polyarthritis — large joints (knees, ankles, wrists, elbows), extremely painful, fleeting
Chest pain and dyspnoea (carditis)
Involuntary jerky movements of hands and face (Sydenham chorea — may present months after other features)
Signs
New cardiac murmur — pansystolic (MR) or early diastolic (AR), pericardial rub
Swollen, hot, tender large joints (migratory pattern — one joint improves as another flares)
Erythema marginatum — pink/red non-pruritic rings on trunk (evanescent, never on face)
Subcutaneous nodules — firm, painless, pea-sized, over bony prominences and extensor tendons
Sydenham chorea: involuntary purposeless rapid movements, emotional lability, muscle weakness
Tachycardia out of proportion to fever
Investigations
First-line
Evidence of recent GAS infectionRaised ASO titre (>200 IU/mL) or anti-DNase B; positive throat swab culture; positive rapid antigen test. At least one is required for Jones criteria
BloodsRaised ESR and CRP (invariably elevated). FBC: leucocytosis, normocytic anaemia
ECGProlonged PR interval (first-degree AV block) — minor criterion. Other conduction abnormalities if carditis severe
Second-line
EchocardiographyAssess for carditis — valvulitis (MR most common, AR), pericardial effusion, myocardial dysfunction. Subclinical carditis detectable on echo may count as major criterion in revised Jones criteria
Chest X-rayCardiomegaly if severe carditis or pericardial effusion
Specialist
Cardiac MRIIf echo equivocal — assess myocardial inflammation
1
Acute episode
- Bed rest until inflammatory markers normalise
- Eradicate GAS: phenoxymethylpenicillin (penicillin V) 500 mg QDS for 10 days or single dose IM benzathine penicillin G 1.2 MU
- Arthritis: aspirin (high-dose, 80–100 mg/kg/day in children) or naproxen. Arthritis responds dramatically within 24–48 h
- Mild carditis: aspirin or NSAIDs
- Severe carditis (heart failure, pericarditis): prednisolone 1–2 mg/kg/day, tapered over 6–12 weeks
- Sydenham chorea: symptomatic — valproate or carbamazepine if severe. Usually self-limiting over months
2
Secondary prophylaxis — prevent recurrence
- IM benzathine penicillin G 1.2 MU every 3–4 weeks (most effective) or oral penicillin V 250 mg BD
- Duration: no carditis = 5 years or until age 21 (whichever longer); carditis without valve disease = 10 years or until age 21; persistent valve disease = lifelong or until age 40
- Penicillin allergy: erythromycin 250 mg BD
Complications
- Rheumatic heart disease: Permanent valvular damage — most commonly mitral stenosis (years-decades later), also MR, AR
- Heart failure: Acute (from severe carditis) or chronic (from valve damage)
- Recurrence: Each recurrence worsens valve damage — prophylaxis is critical
- Atrial fibrillation: Secondary to LA dilatation from mitral valve disease
UKMLA Exam Tips
- 1Jones criteria mnemonic for major: J♥NESS — Joint (polyarthritis), ♥ (carditis), Nodules (subcutaneous), Erythema marginatum, Sydenham chorea
- 2Arthritis in RF is migratory and fleeting — it never causes permanent joint damage
- 3Carditis is the ONLY manifestation that causes permanent damage (rheumatic heart disease)
- 4Chorea may appear months after the acute episode — it is the only manifestation that can occur in isolation and still satisfy Jones criteria
- 5Erythema marginatum = on trunk, NEVER on face; subcutaneous nodules = on extensor surfaces/bony prominences
- 6Secondary prophylaxis with penicillin prevents recurrence — duration depends on presence and severity of cardiac involvement
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