📌 Jones Criteria (Revised 2015) – Major CriteriaCarditis (clinical or subclinical by echo), polyarthritis, chorea, erythema marginatum, subcutaneous nodules.
📌 Jones Criteria – Minor CriteriaArthralgia, fever (>38.5°C), elevated ESR/CRP, prolonged PR interval on ECG.
📌 Evidence of preceding Group A Strep infectionThroat culture positive for GAS, elevated ASO titer, or anti-DNase B. Required for diagnosis of first episode of ARF (except chorea or indolent carditis).
📌 Carditis in ARFMitral regurgitation (most common), aortic regurgitation. May present as new murmur, cardiomegaly, heart failure, pericarditis. Subclinical carditis on echo without murmur is major in high-risk populations.
📌 Sydenham choreaLate manifestation (1-6 months after strep). Involuntary movements, emotional lability, hypotonia. Self-limited, may require symptomatic treatment (valproate, haloperidol).
📌 Secondary prophylaxis – Benzathine penicillin GIM every 3-4 weeks (weight-adjusted). Duration: carditis with residual RHD → 10 years or until age 40 (whichever longer). No carditis → 5 years or until age 21.
📌 Echocardiographic criteria for RHD (World Heart Federation)Mitral regurgitation (pathologic), aortic regurgitation, morphological changes (leaflet thickening, chordal elongation). Used for screening in endemic regions.
📌 Management of acute rheumatic carditisBed rest, aspirin (50-60 mg/kg/day) for arthritis/arthralgia. Corticosteroids for severe carditis with heart failure. ACE inhibitors/ diuretics for heart failure. Treat strep (penicillin 10 days).
📌 Chronic RHD – valve complicationsMitral stenosis (years later), combined MS+MR, aortic stenosis/regurgitation. May need balloon valvuloplasty (mitral) or valve replacement. Anticoagulation for mechanical valves.
📌 Transition & RHD in ACHDLifelong secondary prophylaxis. Pregnancy: high risk if moderate-severe valve disease (mitral stenosis, aortic stenosis). Pre-conception balloon mitral valvuloplasty may reduce risk.
📈 Step 2: Elicit history of preceding sore throat Group A strep pharyngitis 1-5 weeks prior. Throat culture, ASO, anti-DNase B titers.
🩺 Step 3: Apply Jones criteria (2 major or 1 major + 2 minor + evidence of strep) Low-risk vs moderate/high-risk populations (echocardiographic subclinical carditis is major in high-risk).
🔄 Step 4: Echocardiogram for carditis All suspected ARF should have echo to detect valvular regurgitation/stenosis, even if no murmur.
🚨 Step 5: Differentiate from other arthritis JIA, septic arthritis, reactive arthritis, post-streptococcal reactive arthritis (no carditis).
1️⃣ Acute phase – Treat strep infection ▪ Benzathine penicillin G 600,000-1,200,000 units IM once (or oral penicillin V 250 mg tid ×10 days). ▪ Erythromycin if penicillin-allergic.
2️⃣ Anti-inflammatory therapy ▪ Arthritis/arthralgia: aspirin 50-60 mg/kg/day (max 4 g/day) ×2-4 weeks. ▪ Severe carditis with heart failure: prednisone 1-2 mg/kg/day for 2 weeks, taper over 2 weeks. ▪ NSAIDs (naproxen) alternative but avoid in heart failure.
3️⃣ Manage heart failure ▪ Diuretics (furosemide), ACE inhibitors, digoxin (if atrial fibrillation). ▪ Bed rest until improvement, then gradual mobilization.
4️⃣ Manage chorea ▪ Reassurance, protective environment. ▪ Valproic acid or carbamazepine for severe cases. ▪ Haloperidol if refractory (risk of EPS).
5️⃣ Secondary prophylaxis (prevent recurrence) ▪ Benzathine penicillin G IM: — ≤27 kg: 600,000 U every 4 weeks. — >27 kg: 1,200,000 U every 4 weeks (q3 weeks in high-risk). ▪ Duration: carditis with residual RHD → 10 years or until age 40 (whichever longer). No carditis → 5 years or until age 21.
6️⃣ Chronic RHD – valve intervention ▪ Mitral stenosis: balloon valvuloplasty if favorable anatomy. ▪ Mitral/aortic regurgitation with symptoms or LV dysfunction: valve repair or replacement. ▪ Mechanical valves require lifelong warfarin (target INR 2.5-3.5 for mitral, 2.0-3.0 for aortic).
7️⃣ Transition & pregnancy ▪ High-risk pregnancy if mitral stenosis (MVA <1.5 cm²) or severe aortic stenosis. ▪ Pre-conception balloon valvuloplasty. ▪ Avoid warfarin in first trimester (teratogenic); use heparin or LMWH.
❓ Reflex prompt 1: A 9-year-old with migratory polyarthritis (knees, ankles), fever, and positive ASO. No murmur. Jones criteria: 1 major + 2 minor + strep = definite ARF. Next step? ✅ Answer: Echocardiogram to rule out subclinical carditis. Start aspirin for arthritis, benzathine penicillin for strep, then secondary prophylaxis for 5 years.
❓ Reflex prompt 2: A 12-year-old with ARF and severe mitral regurgitation, heart failure. Treatment? ✅ Answer: Corticosteroids (prednisone) for severe carditis, diuretics, ACE inhibitors. Surgical valve repair if refractory failure.
❓ Reflex prompt 3: A 14-year-old with chorea, emotional lability, normal echo, ASO elevated. Best therapy? ✅ Answer: Chorea is self-limited. Avoid stress. Valproic acid or carbamazepine if disabling. Secondary prophylaxis required despite no carditis.
❓ Reflex prompt 4: A 25-year-old with history of ARF and mitral stenosis (valve area 1.0 cm²) wants to become pregnant. Recommendation? ✅ Answer: High-risk pregnancy. Pre-conception balloon mitral valvuloplasty to increase valve area (>1.5 cm²). Avoid warfarin in first trimester.
❓ Reflex prompt 5: A 16-year-old with RHD and mechanical mitral valve on warfarin. Transition to adult care should include:? ✅ Answer: Lifelong anticoagulation (INR target 2.5-3.5), secondary prophylaxis (benzathine penicillin) until age 40, pregnancy counselling, avoidance of contact sports.
❓ Reflex prompt 6: A child with ARF and erythema marginatum on trunk. How long does this rash last? ✅ Answer: Transient, non-pruritic, often appears and disappears within hours. Not painful.
1. Etiology: Post-streptococcal autoimmune complication of group A β-hemolytic streptococcus (GAS) pharyngitis. Not following skin infections. 2. Epidemiology: Highest burden in low/middle-income countries (India, Africa, Pacific). In endemic areas, subclinical carditis detected by echocardiography. 3. Jones Criteria (Modified 2015) – Diagnosis of first episode ARF:
- 2 major, OR 1 major + 2 minor + evidence of preceding GAS infection.
- Major criteria: carditis (clinical or subclinical on echo), polyarthritis, chorea, erythema marginatum, subcutaneous nodules.
- Minor criteria: arthralgia, fever (≥38.5°C), elevated ESR/CRP, prolonged PR interval.
- Evidence of GAS: positive throat culture, elevated ASO/anti-DNase B. 4. Carditis: Most serious manifestation (mitral regurgitation > aortic regurgitation). Echocardiogram mandatory. Subclinical carditis is major in moderate/high-risk populations. 5. Acute management:
- Treat GAS infection (benzathine penicillin IM once).
- Arthritis: aspirin 50-60 mg/kg/day.
- Severe carditis + heart failure: prednisone 1-2 mg/kg/day; diuretics, ACE inhibitors.
- Chorea: valproate, carbamazepine (severe cases). 6. Secondary prophylaxis (prevent recurrence):
- Benzathine penicillin G IM every 4 weeks (q3 weeks in high-risk).
- Duration: carditis with residual RHD → 10 years or until age 40 (whichever longer). No carditis → 5 years or until age 21. 7. Chronic RHD: Mitral stenosis (late), combined lesions. Balloon mitral valvuloplasty (MV area >1.5 cm²). Valve replacement for severe symptomatic disease. Mechanical valves require anticoagulation (INR 2.5-3.5 for mitral). 8. Transition & pregnancy: High-risk pregnancy if moderate-severe mitral stenosis (MVA <1.5 cm²) or aortic stenosis. Pre-conception intervention advised. 💡 Clinical pearls:
➤ Echocardiogram should be performed in all suspected ARF, even if no murmur (subclinical carditis).
➤ Sydenham chorea may be the only manifestation of ARF; requires secondary prophylaxis despite normal echo.
➤ Recurrence of ARF increases risk of severe RHD; strict adherence to secondary prophylaxis is essential.
➤ In endemic regions, echocardiographic screening of schoolchildren identifies early RHD for secondary prophylaxis.