Treatment of Rheumatic Fever Flare
For a patient experiencing a rheumatic fever flare, immediately initiate a full 10-day course of penicillin to eradicate Group A Streptococcus, followed by long-term secondary prophylaxis with intramuscular benzathine penicillin G 1.2 million units every 4 weeks, and add anti-inflammatory therapy with aspirin or corticosteroids for symptomatic relief. 1, 2, 3
Acute Treatment of the Flare
Antibiotic Therapy to Eradicate Streptococcus
Administer a full therapeutic course of penicillin for 10 days to eradicate any residual Group A Streptococcus, even if the throat culture is negative at the time of diagnosis. 1, 3, 4
For adults and children ≥40 kg: Penicillin V 500 mg orally 2-3 times daily for 10 days 1, 4
For children <40 kg: Penicillin V 250 mg orally twice daily for 10 days 1, 4
For penicillin-allergic patients: Azithromycin 500 mg once daily for 5 days or clarithromycin 250 mg twice daily for 10 days (adults); erythromycin or first-generation cephalosporins if no immediate-type hypersensitivity (children) 1, 3
Anti-Inflammatory Therapy for Symptom Control
For arthritis without severe carditis: Use aspirin as first-line anti-inflammatory therapy. 1, 5
For severe carditis or cardiac involvement: Consider corticosteroids such as prednisone 1-2 mg/kg/day for 1-2 weeks. 1, 5
In cases with severe pericarditis or significant cardiac involvement, intravenous methylprednisolone 1000 mg/day initially may be considered, followed by oral prednisone 1
Evidence shows that corticosteroids versus aspirin do not significantly reduce the risk of cardiac disease at one year (RR 0.87,95% CI 0.66 to 1.15), so the choice should be based on severity of acute symptoms rather than expectation of preventing long-term valve damage 5
Naproxen is a safe and effective alternative to aspirin in children, with lower hepatotoxicity rates 6
Long-Term Secondary Prophylaxis (Critical for Preventing Recurrence)
First-Line Prophylactic Regimen
Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the gold standard for secondary prophylaxis and is approximately 10 times more effective than oral antibiotics. 3, 1, 2, 7, 8
For high-risk patients (those with recurrences despite adherence, severe valvular disease, or high streptococcal exposure): Administer benzathine penicillin G every 3 weeks instead of every 4 weeks to maintain more consistent protective levels 3, 1, 2, 7
Intramuscular penicillin reduces rheumatic fever recurrence substantially compared to oral antibiotics (RR 0.07,95% CI 0.02 to 0.26), meaning only 0.1% recurrence with IM versus 1% with oral 8
Alternative Regimens for Penicillin Allergy
Oral penicillin V 250 mg twice daily (second-line option, but substantially less effective than intramuscular) 3, 1, 2, 7
Sulfadiazine 1 gram orally once daily (or 0.5 gram for patients ≤27 kg) for penicillin-allergic patients 3, 1, 2, 7
Macrolide or azalide antibiotics for patients allergic to both penicillin and sulfadiazine, but avoid in patients taking cytochrome P450 3A inhibitors due to QT prolongation risk 3, 1, 7
Duration of Secondary Prophylaxis
The duration depends on the severity of cardiac involvement and must be individualized:
Patients with rheumatic carditis and residual valvular heart disease: Continue prophylaxis for at least 10 years after the last attack OR until age 40 years (whichever is longer), often lifelong. 3, 1, 2, 7
Patients with rheumatic carditis but no residual heart disease: Continue for 10 years after the last attack OR until age 21 years (whichever is longer) 3, 1, 2, 7
Patients with rheumatic fever without carditis: Continue for 5 years after the last attack OR until age 21 years (whichever is longer) 3, 1, 2, 7
Critical Pitfalls to Avoid
Never discontinue secondary prophylaxis prematurely, even if the patient feels well or has undergone valve surgery—prophylaxis must continue even after valve replacement because surgery does not eliminate the risk of recurrent acute rheumatic fever from Group A Streptococcus infection. 1, 2, 7
At least one-third of rheumatic fever cases result from asymptomatic Group A Streptococcus infections, making prevention challenging and underscoring the absolute necessity of continuous prophylaxis 1, 2
Recurrent rheumatic fever is associated with worsening of rheumatic heart disease, and infection does not have to be symptomatic to trigger a recurrence 3
Do not confuse secondary prophylaxis for rheumatic fever with infective endocarditis prophylaxis—current guidelines do not recommend routine endocarditis prophylaxis for rheumatic heart disease alone, only for patients with prosthetic valves, prosthetic material used in valve repair, or previous infective endocarditis 3, 2, 7
Additional Management Considerations
Maintain optimal oral health as the most important preventive measure against infective endocarditis 3, 2, 7
Administer influenza and pneumococcal vaccinations according to standard recommendations 3, 2, 7
If left ventricular systolic dysfunction develops, apply guideline-directed medical therapy including ACE inhibitors or ARBs and beta-blockers, but avoid abrupt lowering of blood pressure in patients with stenotic valve lesions 3, 2
Family members of patients with current or previous rheumatic fever should have prompt treatment of any streptococcal infections 1