What is the management of acute rheumatic fever?

Management of Acute Rheumatic Fever

Acute rheumatic fever management requires three simultaneous interventions: complete eradication of Group A Streptococcus with a full 10-day course of penicillin, immediate initiation of long-term secondary prophylaxis with intramuscular benzathine penicillin G, and anti-inflammatory therapy with high-dose aspirin for symptomatic relief. 1, 2

Acute Phase Antibiotic Treatment

Primary Treatment to Eradicate GAS

A full 10-day course of penicillin must be administered to eradicate residual Group A Streptococcus, even if the throat culture is negative at the time of diagnosis. 1, 2 This is critical because treatment can prevent further cardiac complications even when started up to 9 days after symptom onset. 1

First-line options:

• Oral Penicillin V: 250 mg twice daily for children <27 kg; 500 mg 2-3 times daily for children ≥27 kg, adolescents, and adults, all for 10 days 1, 2
• Intramuscular Benzathine Penicillin G: Single injection of 600,000 units for patients <27 kg; 1,200,000 units for patients ≥27 kg 1

When to strongly favor intramuscular over oral therapy:

• Patients unlikely to complete a 10-day oral course 1
• Personal or family history of rheumatic fever or rheumatic heart disease 1
• Environmental risk factors (poverty, overcrowding) 1
• Unreliable follow-up 1

For penicillin-allergic patients:

• Erythromycin or first-generation cephalosporins (if no immediate-type hypersensitivity) 2
• Azithromycin 500 mg once daily for 5 days or clarithromycin 250 mg twice daily for 10 days in adults 2

Important caveat: Warming benzathine penicillin G to room temperature before administration reduces discomfort. 1 Patients become non-contagious after 24 hours of antibiotic therapy. 1

Secondary Prophylaxis (Prevention of Recurrence)

Continuous antimicrobial prophylaxis must be initiated immediately upon diagnosis of acute rheumatic fever—this is the most critical intervention to prevent recurrent attacks and progressive cardiac damage. 1, 2, 3

Gold Standard Regimen

Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the gold standard, providing approximately 10 times greater protection than oral antibiotics (0.1% vs 1% recurrence rate). 2, 3, 4 This represents a relative risk reduction of 0.07 (95% CI 0.02 to 0.26) compared to oral antibiotics. 2

For high-risk populations or patients with recurrence despite adherence to the 4-week regimen, administer benzathine penicillin G every 3 weeks. 1, 2, 3 High-risk populations include children, adolescents, parents of young children, teachers, healthcare workers, military recruits, and economically disadvantaged populations. 3

Duration of Secondary Prophylaxis

The duration depends entirely on the presence and severity of cardiac involvement during the acute episode:

• Rheumatic carditis WITH residual heart disease (persistent valvular disease): At least 10 years after the last episode OR until age 40 years (whichever is longer), often lifelong 1, 2, 3

• Rheumatic carditis WITHOUT residual heart disease (normal valvular function on echocardiography): 10 years after the last attack OR until age 21 years (whichever is longer) 2, 3

• No carditis: 5 years after the last attack OR until age 21 years (whichever is longer) 2, 3

Critical pitfall to avoid: Never discontinue prophylaxis prematurely based solely on normal echocardiographic findings, as patients remain susceptible to Group A Streptococcus infection and at least one-third of rheumatic fever cases arise from asymptomatic streptococcal infections. 3 Prophylaxis should continue even after valve surgery, including prosthetic valve replacement. 2

Alternative Prophylaxis Regimens

For penicillin-allergic patients:

• Erythromycin orally twice daily 4
• Sulfadiazine 5, 6

Important warning: Avoid prescribing macrolides with cytochrome P450 3A inhibitors due to QT prolongation risk. 2

Anti-Inflammatory Treatment for Symptomatic Relief

Arthritis and Mild Carditis

High-dose aspirin (acetylsalicylic acid) 75-100 mg/kg/day is effective for controlling inflammatory manifestations of arthritis and mild carditis. 1 The arthritis of rheumatic fever responds rapidly to aspirin, typically resolving within days of initiating therapy. 1

Treatment duration: Continue for 4-6 weeks. 1

Severe Carditis or Cardiac Involvement

For severe inflammation or significant cardiac involvement, corticosteroids such as prednisone 1-2 mg/kg/day for 1-2 weeks may be considered. 2 In severe cases with significant cardiac involvement, intravenous methylprednisolone (1000 mg/day initially) may be considered, followed by oral prednisone. 2

Important evidence limitation: A Cochrane review of eight randomized controlled trials involving 996 patients found no significant difference in risk of cardiac disease at one year between corticosteroid-treated and aspirin-treated groups (risk ratio 0.87,95% CI 0.66 to 1.15). 7 However, these studies were conducted between 1950-2001 with substantial risk of bias, so results should be interpreted with caution. 7

The key distinction: Monthly intramuscular penicillin represents secondary prophylaxis to prevent recurrent attacks, NOT treatment of the acute symptomatic episode—anti-inflammatory therapy is needed separately for symptom control. 1

Monitoring and Follow-Up

Serial echocardiographic evaluations are essential for monitoring patients with previous rheumatic fever, as they can detect subclinical progression of valvular disease not apparent on physical examination. 3 Echocardiography confirms normal valvular function and documents the absence of mitral regurgitation. 3

Endocarditis Prophylaxis

Routine endocarditis prophylaxis is NOT recommended for patients with rheumatic heart disease receiving benzathine penicillin G prophylaxis, UNLESS they have prosthetic valves or prosthetic material used in valve repair. 3

For high-risk dental or surgical procedures in patients with rheumatic heart disease:

• Amoxicillin prophylaxis before the procedure 4
• If recently treated with penicillin/amoxicillin or have immediate penicillin hypersensitivity, use clindamycin 4

Family Considerations

Family members of patients with current or previous rheumatic fever should receive prompt treatment of any streptococcal infections. 2