Treatment of Rheumatic Fever and Rheumatic Heart Disease

Acute Rheumatic Fever (ARF)

1. Eradication of Group A Streptococcus (Primary Step)

• Benzathine penicillin G (BPG) IM - single dose

  • 27 kg (60 lb): 1.2 million units IM

  • <27 kg: 600,000 units IM

• Penicillin V 250 mg twice daily x 10 days
• Amoxicillin 50 mg/kg once daily (max 1 g) x 10 days
• Azithromycin 250 mg daily (for penicillin-allergic patients)
• Erythromycin 40 mg/kg/day (max 1 g/day) divided 2-4 doses x 10 days

Goldman-Cecil Medicine, 2-Volume Set

2. Anti-inflammatory Therapy

For Arthritis and Fever

• Aspirin - first-line; 80-100 mg/kg/day in children (4-8 g/day in adults), in divided doses
• Naproxen 10-20 mg/kg/day divided twice daily - used with success in small studies as an alternative
• NSAIDs should continue for 1-2 weeks until ALL symptoms have resolved (not just joint symptoms)

Goldman-Cecil Medicine; Firestein & Kelley's Textbook of Rheumatology

For Carditis (Severe)

• Prednisone 1-2 mg/kg/day (max 80 mg/day, once daily or divided) is the glucocorticoid of choice
• After 2-3 weeks, taper by 20-25% weekly
• The 2020 Australian guidelines list glucocorticoids as optional for very severe carditis; high-quality evidence is still limited
• IV methylprednisolone is recommended by WHO and NZ/Australian guidelines in life-threatening situations
• After stopping glucocorticoids, resume NSAIDs to prevent rebound fever and joint symptoms
• IVIG was not shown to alter the natural history of ARF and is NOT recommended

Firestein & Kelley's Textbook of Rheumatology, 2-Volume Set

For Heart Failure (due to carditis)

• Diuretics
• Angiotensin receptor blockers (ARBs)
• Fluid restriction and bed rest
• Beta-blockers are not currently recommended for HF due to acute carditis
• Valve surgery is rarely needed in acute ARF, but can be helpful in cases of acute valve leaflet rupture

For Sydenham's Chorea

• Usually resolves within weeks to months without treatment
• Valproic acid or carbamazepine can be used for severe or persistent cases
• Haloperidol (low dose) has also been used in refractory chorea

3. Bed Rest

• Strict bed rest is no longer universally recommended
• Normalization of ESR and CRP is used as a practical guide for return to exercise

Secondary Prophylaxis (Preventing Recurrence)

Drug of Choice:

IM BPG is more effective than oral penicillin for preventing recurrences.

Duration of Secondary Prophylaxis:

Firestein & Kelley's Textbook of Rheumatology; Goldman-Cecil Medicine

Rheumatic Heart Disease (RHD)

Medical Management:

• Continue secondary prophylaxis (BPG as above) - this is the cornerstone
• Echocardiography for diagnosis, follow-up, and surveillance
• Atrial fibrillation (a frequent complication) - rate control and anticoagulation (warfarin preferred over NOACs in RHD-associated AF)
• Heart failure - standard HF therapy (diuretics, ACE inhibitors/ARBs, beta-blockers)
• Infective endocarditis prophylaxis - per current guidelines for high-risk valve lesions

Interventional/Surgical Management:

• Percutaneous mitral balloon valvotomy (PMBV) - procedure of choice for suitable mitral stenosis (pliable, non-calcified valve, no significant MR)
• Mitral valve repair or replacement - for severe mitral regurgitation or calcified/unsuitable stenotic valves
• Aortic valve repair or replacement - for significant aortic regurgitation or stenosis
• Catheter-based interventions are the first consideration per current valvular disease guidelines

Goldman-Cecil Medicine; Firestein & Kelley's Textbook of Rheumatology; Sabiston Textbook of Surgery

Summary Table: ARF Treatment at a Glance