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Cardiovascular

Rheumatic Fever (RF)



Diagnostic criteria

  • Evidence of recent Streptococcus pyogenes infection (throat swab or elevated anti-streptolysin O titres/anti-DNase B)

  • 2 major criteria OR 1 major + 2 minor criteria


JONES PEACE


Major criteria (JONES):

  • J: Joints (polyarthritis, hot/swollen joints)

  • O: Heart (carditis: endocarditis/myocarditis/pericarditis, murmurs)

  • N: Nodules (subcutaneous, painless)

  • E: Erythema marginatum (non-itchy, pink, ring-like rash)

  • S: Sydenham chorea (involuntary movements, emotional lability)


Minor criteria (PEACE):

  • P: PR prolongation (on ECG)

  • E: Elevated ESR >30 mm/hr

  • A: Arthralgia (joint pain without swelling)

  • C: CRP elevation >30 mg/L

  • E: Elevated temperature (fever ≥38°C)


Diagnosis

  • Blood tests: FBC, ESR, CRP

  • Infectious evidence: Streptococcal ASOT and anti-DNase B titres, throat swab MCS

  • Imaging:

    • ECG: Look for PR prolongation, signs of carditis

    • Echocardiogram: Essential even in the absence of symptoms, particularly to identify subclinical carditis

    • CXR: Assess for cardiomegaly in suspected severe carditis


Treatment


Short term:

  • Antibiotics: IM benzathine benzylpenicillin (same dose as for streptococcal throat infections)

    • For penicillin-allergic patients: Cephalexin or azithromycin (duration per guidelines)

  • Symptomatic relief:

    • Arthritis: Ibuprofen (10 mg/kg TDS) or paracetamol (15 mg/kg QID)

    • Severe arthritis/arthralgia: Aspirin (continue until symptom-free for 1–2 weeks, usually for 6 weeks)

  • Bed rest: Until CRP normalises


Long term:

  • Secondary prophylaxis: IM benzathine benzylpenicillin monthly

    • Duration:

      • 10 years post-episode or until age 21 (whichever is later)

      • Until age 35 for moderate rheumatic heart disease (RHD)

      • Until age 40 or lifelong for severe RHD or post-cardiac valve surgery


Note:

  • Perform an echocardiogram even in asymptomatic cases to detect subclinical carditis

  • In patients with a previous history of RF, diagnosis may be based on 3 minor criteria alone


Causes of elevated RF other than RA

  • Normal variant

  • Infections: Hepatitis, tuberculosis, Epstein-Barr virus (EBV)

  • Autoimmune/connective tissue diseases: SLE, Sjögren’s syndrome, systemic sclerosis

  • Liver cirrhosis

  • Sarcoidosis

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