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|Congenital Cyanotic Heart Disease
|Cardiac Embryology
|Cyanosis - Central and Peripheral
|Down's syndrome (Trisomy 21)
|Tetralogy of Fallot
|Patent Foramen Ovale (PFO)
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|Mitral Stenosis vs Regurgitation
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|Transcatheter aortic valve implantation (TAVI)
|Infective Endocarditis
| ⚡ Acute Rheumatic Fever (ARF) |
- Diagnosis is based on the Modified Jones Criteria ⚖️.
- ⏳ Onset 2–3 weeks after Group A Streptococcus pharyngitis 🦠.
- Flitting arthritis 🤕 and new murmurs ❤️ are classical.
- Antibiotics: Penicillin or Amoxicillin (alternatives: azithromycin, cephalexin).
- High-dose Aspirin 💊 is traditionally used for arthritis.
- Early treatment reduces long-term rheumatic heart disease risk.
- Requires lifelong cardiac monitoring ❤️🩹 for valvular damage.
|
Rheumatic fever is an inflammatory disease occurring as a delayed sequel to
pharyngeal infection with group A streptococci. MacCallum’s Plaque is 🧾 typically at base of posterior mitral leaflet. Caused by subendocardial Aschoff bodies (necrotic core + lymphocytes). These lesions heal with extensive myocardial fibrosis.
📖 Introduction
- Autoimmune condition triggered by Streptococcus pyogenes 🦠.
- Involves heart, joints, CNS, skin and subcutaneous tissues.
- Molecular mimicry → antibodies cross-react with cardiac myosin & sarcolemmal proteins.
- Pancarditis: endocarditis, myocarditis & pericarditis.
- Exudative and proliferative inflammatory lesions of the connective tissues.
🌍 Epidemiology
- Common cause of acquired heart disease in low-income countries.
- Declining incidence in Europe/North America over past 4–6 decades.
- High burden in Pacific, South Asia, Sub-Saharan Africa.
- Low-risk = incidence < 2 per 100,000 children (5–14y).
🧬 Aetiology
- Triggered by GAS pharyngitis (not skin infection).
- Immune cross-reactivity → Aschoff nodules (granulomatous inflammation).
- Valve damage → stenosis + regurgitation (mitral > aortic > tricuspid > pulmonary).
🩺 Clinical Features
- Fever, lethargy, anorexia.
- ❤️ Pancarditis → new murmurs (Carey Coombs mid-diastolic), Apical systolic murmur, pericarditis, cardiomegaly, Congestive heart failure.
- 🦵 Migratory polyarthritis → knees, ankles, elbows, wrists.
- 🌸 Erythema marginatum → pink rings on trunk/limbs.
- 📍 Subcutaneous nodules → painless, extensor surfaces.
- 🧠 Sydenham chorea → emotional lability + jerky movements (late).
🧪 Investigations
- FBC/ESR/CRP → raised. These indicate systemic inflammation and are useful for
monitoring progress of the disease.
- ECG → prolonged PR, arrhythmias may reveal evidence of carditis.
- CXR → cardiomegaly, pulmonary oedema.
- Echo → valve damage, dilatation may reveal evidence of carditis.
- ASO titre ↑ (evidence of strep).
⚖️ Jones Criteria (Revised)
Diagnosis = 2 major OR 1 major + 2 minor, + evidence of recent GAS infection.
| Major Criteria | Minor Criteria |
|---|
- Carditis ❤️
- Polyarthritis 🤕
- Chorea 🧠
- Erythema marginatum 🌸
- Subcutaneous nodules 📍
|
- Arthralgia
- Fever 🌡️
- ↑ ESR/CRP 📈
- Prolonged PR on ECG
|
💊 Management
- Bed rest lessens joint pain and reduces cardiac workload + supportive care.
- Aspirin usually at high weight adjusted doses/ NSAIDs for arthritis.
- Steroids e.g Prednisolone 1-2 mg/kg (severe carditis). It produces more rapid symptomatic relief and is indicated
for carditis or severe arthritis, until the ESR returns to normal.
- Anticonvulsants (valproate, carbamazepine) for chorea.
- Patients are susceptible to further attacks of rheumatic fever if subsequent
streptococcal infection occurs, and long-term prophylaxis with penicillin should be
given, usually until the age of 21.
🚑 Eradication & Prophylaxis
- Acute episode: Penicillin V (10 days) or IM benzathine penicillin. Alternatives = erythromycin.
- Secondary prophylaxis (to prevent recurrence):
- No carditis → 5y or until age 21.
- Carditis, no residual disease → 10y or until 21.
- Severe carditis with valve disease → ≥10y, sometimes lifelong.
