7.4. Rheumatic Fever (and Rheumatic Heart Disease)

📄 Definition

Rheumatic fever is an autoimmune inflammatory condition that occurs after Group A Streptococcal (GAS) pharyngitis. It can cause permanent heart valve damage — known as rheumatic heart disease (RHD).

🧫 Pathophysiology

  • Molecular mimicry: Antibodies against GAS M-proteins cross-react with cardiac tissue.

  • Multisystem inflammatory response (especially joints, heart, skin, brain).

🧠 Diagnostic Criteria: Modified Jones Criteria (2023 update)

Diagnosis = Evidence of recent GAS infection +

  • 2 major criteria, or

  • 1 major + 2 minor criteria

🟥 Major Criteria – Mnemonic: JONES

  • Joints – migratory polyarthritis (large joints)

  • O♥️ – carditis (pancarditis, new murmur)

  • Nodules – subcutaneous, firm, painless

  • Erythema marginatum – annular rash

  • Sydenham’s chorea – rapid, involuntary movements

🟨 Minor Criteria

  • Fever ≥38.5°C

  • Raised ESR/CRP

  • Prolonged PR interval on ECG

  • Arthralgia

🧪 Evidence of GAS Infection

  • Positive throat swab culture or rapid antigen test

  • Raised/rising ASO or anti-DNase B titre

⚠️ Clinical Features

  • 2–4 weeks post-streptococcal pharyngitis

  • Polyarthritis, carditis (tachycardia, murmurs), chorea, rash

  • New murmur = mitral regurgitation most common

Investigations

🥇 First-Line

  • Throat swab – culture or rapid GAS test

  • FBC, CRP/ESR – inflammation

  • ECG – PR prolongation

  • ASO titres – past GAS infection

🥈 Second-Line

  • Echocardiogram – assess valve involvement

  • CXR – cardiomegaly if carditis

  • MRI Brain – if chorea present

Management

🔴 Acute Rheumatic Fever

  • Penicillin V – 10-day course for GAS eradication

  • NSAIDs (aspirin/naproxen) – for arthritis

  • Corticosteroids – if severe carditis or chorea

  • Supportive care – bed rest during acute phase

🛡️ Secondary Prophylaxis (Rheumatic Heart Disease)

  • Benzathine penicillin IM every 21–28 days

  • Continue for:

    • 5 years or until 21 years old (whichever longer) – no carditis

    • 10 years or until 40 years old – with carditis but no RHD

    • Lifetime – if persistent RHD

🔺 Last updated in line with NICE CKS and WHO/WHF RHD guidelines
Published: February 2022 • Last updated: November 2023

  • PARA-aligned, reviewed February 2026


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