Rheumatic Fever: Comprehensive Management
Primary Treatment of Acute Rheumatic Fever
All patients with acute rheumatic fever must receive a full therapeutic course of penicillin to eradicate residual Group A Streptococcus, even if throat culture is negative at the time of diagnosis. 1, 2
Acute Phase Antibiotic Therapy
- Penicillin V potassium: 250 mg orally twice daily for children or 500 mg 2-3 times daily for adolescents/adults for 10 days 3, 4
- Amoxicillin alternative: 500 mg every 12 hours or 250 mg every 8 hours for mild/moderate infections; 875 mg every 12 hours or 500 mg every 8 hours for severe infections 4
- Treatment must continue for at least 10 days to prevent acute rheumatic fever recurrence 4
Symptomatic Management
- Acetaminophen or NSAIDs for moderate to severe symptoms or high fever 2, 5
- Avoid aspirin in children due to Reye's syndrome risk 2, 5
Secondary Prophylaxis: The Cornerstone of Long-Term Management
Continuous antimicrobial prophylaxis is mandatory for all patients with a history of rheumatic fever or rheumatic heart disease, as recurrence can occur even with optimal treatment of symptomatic infections and can worsen valvular damage. 1, 2
Prophylaxis Regimens (in order of preference)
First-line (Gold Standard):
- Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks (Class I, Level A evidence) 1, 2
- This regimen is approximately 10 times more effective than oral antibiotics 2
- For high-risk patients (those with recurrence despite adherence, severe valvular disease, or high Group A Streptococcus exposure): administer every 3 weeks instead of every 4 weeks 1, 2, 5
Oral alternatives (for patients who refuse or cannot tolerate IM injections):
- Penicillin V potassium: 250 mg orally twice daily 1, 2, 3
- Sulfadiazine: 1 g orally once daily (adults) or 0.5 g once daily for patients ≤27 kg 1, 2
For penicillin allergy:
- Macrolide or azalide antibiotics (dose varies by agent) 1, 2
- Critical caveat: Avoid macrolides in patients taking cytochrome P450 3A inhibitors (azole antifungals, HIV protease inhibitors, some SSRIs) 1, 2
Duration of Secondary Prophylaxis
The duration depends on cardiac involvement and residual valvular disease:
Rheumatic Fever WITH Carditis AND Residual Heart Disease
- 10 years after last attack OR until age 40 years, whichever is longer 1, 2, 5
- Consider lifelong prophylaxis for patients at high risk of Group A Streptococcus exposure (teachers, healthcare workers, parents of young children, military recruits, those in crowded living situations) 1, 2, 5
Rheumatic Fever WITH Carditis but NO Residual Heart Disease
Rheumatic Fever WITHOUT Carditis
Management of Cardiac Complications
Heart Failure from Valvular Disease
Apply guideline-directed medical therapy when left ventricular systolic dysfunction develops:
- ACE inhibitors or ARBs 1, 2, 5
- Beta-blockers 1, 2, 5
- Diuretics 2, 5
- Aldosterone antagonists 2, 5
- Sacubitril/valsartan 2, 5
Critical pitfall: Avoid abruptly lowering blood pressure in patients with stenotic valve lesions 1, 2
Severe Symptomatic Mitral Stenosis
- Evaluate all patients for percutaneous mitral balloon commissurotomy or mitral valve surgery within 3 months of diagnosis 2, 5
Monitoring and Surveillance
Echocardiographic Follow-up
- Mild valvular disease: Every 3-5 years 1, 5
- Moderate valvular disease: Every 1-2 years 1, 5
- Severe valvular disease or dilating left ventricle: Every 6-12 months 1, 5
Echocardiography is significantly more sensitive than auscultation alone for detecting valvular involvement and should be used to identify subclinical carditis 5, 6, 7
Infective Endocarditis Prophylaxis
The American Heart Association no longer recommends routine endocarditis prophylaxis for patients with rheumatic heart disease alone. 2
Exceptions (prophylaxis IS indicated):
- Prosthetic cardiac valves 2
- Prosthetic material used for valve repair 2
- Previous infective endocarditis 2
Important consideration: For patients receiving continuous penicillin prophylaxis for rheumatic fever who require endocarditis prophylaxis for dental procedures, use an agent other than penicillin 2, 3
Additional Preventive Measures
Vaccinations
Oral Health
- Optimal oral health maintenance is the most important component of preventing infective endocarditis 1, 2, 5
Exercise
- Regular aerobic exercise is encouraged to maintain cardiovascular fitness in patients with asymptomatic valvular heart disease 1, 2
- Avoid heavy isometric repetitive training, but resistive training with small free weights is acceptable 1
Critical Clinical Pitfalls
Asymptomatic Infections
- At least one-third of rheumatic fever cases result from asymptomatic Group A Streptococcus infections, making primary prevention challenging 8
- Group A Streptococcus infection does not have to be symptomatic to trigger recurrence 1
Treatment Limitations
- Rheumatic fever can recur even when symptomatic infection is treated optimally, which is why continuous prophylaxis (not episodic treatment) is essential 1, 8
Family Members
- Promptly treat streptococcal infections in family members of patients with current or previous rheumatic fever (Class I, Level B evidence) 1
Resistant Streptococci
- Alpha-hemolytic streptococci relatively resistant to penicillin may be found in patients receiving continuous oral penicillin for secondary prevention 3
Diagnosis
Jones Criteria (Revised 2015)
Diagnosis requires documented Group A Streptococcus infection PLUS:
Major criteria include carditis (including subclinical carditis on echocardiography), polyarthritis (or monoarthritis in moderate-to-high-risk populations), Sydenham's chorea, subcutaneous nodules, and erythema marginatum 6, 9, 7, 10
The 2015 revision introduced subclinical carditis detected by echocardiography and monoarthritis as a major criterion in moderate-to-high-risk populations. 6, 7