Management of Acute Rheumatic Fever
Immediately initiate a full 10-day course of penicillin to eradicate Group A Streptococcus (even if throat culture is negative), followed by long-term secondary prophylaxis with intramuscular benzathine penicillin G 1.2 million units every 4 weeks, which is the most effective regimen for preventing recurrent rheumatic fever. 1, 2, 3
Acute Phase Treatment: Eradication of Streptococcus
Antibiotic Therapy for Acute Episode
- Penicillin V oral: 250 mg twice daily for children or 500 mg 2-3 times daily for adolescents/adults for 10 days 1, 4
- Amoxicillin oral: 500 mg every 12 hours or 250 mg every 8 hours for adults; 25 mg/kg/day divided every 12 hours for children for 10 days 1, 4
- Benzathine penicillin G intramuscular: Single dose of 1.2 million units for adults, 900,000 units for older children, or 300,000-600,000 units for infants and children under 60 lbs 1, 5
- A full therapeutic course must be completed to eradicate residual Group A Streptococcus, even when throat culture is negative at diagnosis 1, 3, 6
For Penicillin-Allergic Patients
- Narrow-spectrum oral cephalosporins (cefadroxil or cephalexin) for 10 days if no immediate-type hypersensitivity 1
- Macrolides (erythromycin or clarithromycin) or azalides (azithromycin) for patients allergic to both penicillin and cephalosporins 1, 2
- Avoid cephalosporins in patients with anaphylactic-type penicillin allergy (up to 10% cross-reactivity) 1
Anti-Inflammatory Treatment
Symptomatic Management
While anti-inflammatory agents provide dramatic clinical improvement for arthritis and fever, they do not prevent the development of rheumatic heart disease or alter long-term cardiac outcomes 7, 8. The evidence for corticosteroids in carditis is weak and does not demonstrate improved long-term prognosis 8.
- Aspirin for arthritis and fever: Dose and duration based on clinical response 9, 7
- Corticosteroids may be considered for severe carditis, but evidence shows no significant reduction in cardiac disease at one year (risk ratio 0.87,95% CI 0.66-1.15) compared to aspirin 8
- Chorea management: Requires aggressive treatment with sedatives, as it is no longer considered benign and self-limited 7
Long-Term Secondary Prophylaxis
First-Line Regimen
Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the gold standard, being approximately 10 times more effective than oral antibiotics (0.1% vs 1% recurrence rate) 2, 6, 5
- For high-risk patients (those with recurrence despite adherence to 4-week regimen, or high streptococcal exposure), administer every 3 weeks 2
- Alternative dosing: 600,000 units every 2 weeks 3, 5
- Administer by deep intramuscular injection in upper outer quadrant of buttock or ventrogluteal site 5
Alternative Oral Regimens (Less Effective)
- Penicillin V oral: 250 mg twice daily for children; 500 mg 2-3 times daily for adolescents/adults 2, 6
- Sulfadiazine oral: 1 gram once daily for adults; 0.5 gram once daily for patients ≤27 kg 2, 6
- Macrolides or azalides for patients allergic to both penicillin and sulfonamides 2
Duration of Prophylaxis Based on Cardiac Involvement
With carditis and residual heart disease (persistent valvular disease):
- Continue for 10 years after last attack OR until age 40, whichever is longer 2, 6
- Consider lifelong prophylaxis in high-risk patients with severe valvular disease and high streptococcal exposure 6
With carditis but no residual heart disease:
Without carditis:
Critical Clinical Considerations
Timing and Initiation
- Initiate long-term antimicrobial prophylaxis immediately upon diagnosis of acute rheumatic fever 2
- Long-term antimicrobial prophylaxis provides the most effective protection against recurrent rheumatic fever 2
High-Risk Populations
- Children and adolescents, parents of young children, teachers, healthcare workers, military recruits (high streptococcal exposure) 2
- Economically disadvantaged populations 2
- Patients with multiple previous attacks (higher recurrence risk) 2
Important Pitfalls to Avoid
- Do not discontinue prophylaxis after valve surgery - patients remain susceptible to Group A Streptococcus infection and recurrent acute rheumatic fever 6
- Do not switch to oral prophylaxis prematurely - only consider switching when patients reach late adolescence/young adulthood AND have remained free of rheumatic attacks for at least 5 years 2
- At least one-third of rheumatic fever cases arise from asymptomatic Group A Streptococcus infections, making prevention challenging 2, 3
- Even with optimal treatment of streptococcal pharyngitis, rheumatic fever can still occur in susceptible individuals 2, 3
Endocarditis Prophylaxis
- Routine endocarditis prophylaxis is NOT recommended for patients with rheumatic heart disease unless they have prosthetic valves or prosthetic material used in valve repair 2, 6
- For patients receiving penicillin prophylaxis who require endocarditis prophylaxis for dental procedures, use an agent other than penicillin due to likely oral α-hemolytic streptococci resistance 2, 6