Symptoms of Rheumatic Fever
Rheumatic fever presents with a constellation of symptoms including migratory polyarthritis, carditis, chorea, subcutaneous nodules, and erythema marginatum, which occur following group A streptococcal pharyngitis. 1, 2
Clinical Manifestations
Major Manifestations
Migratory Polyarthritis
Carditis
- Can range from mild and transient to severe
- May lead to permanent valvular damage (rheumatic heart disease)
- More common and severe in children than adults 3
- Can manifest as new murmurs, pericarditis, or heart failure
Sydenham's Chorea
- Involuntary, purposeless movements
- Emotional lability
- May appear weeks to months after the streptococcal infection
- More common in females 4
Subcutaneous Nodules
- Firm, painless nodules over bony prominences
- Usually associated with carditis
Erythema Marginatum
- Transient, non-pruritic rash with central clearing and advancing edges
- Typically on trunk and proximal extremities
Minor Manifestations
- Fever
- Arthralgia
- Elevated acute phase reactants (ESR, CRP)
- Prolonged PR interval on ECG
Laboratory Findings
Evidence of Preceding Streptococcal Infection
- Positive throat culture for group A streptococcus
- Elevated or rising antistreptolysin O (ASO) titer
- Positive rapid antigen detection test (RADT) 1
Inflammatory Markers
- Markedly elevated erythrocyte sedimentation rate (ESR) often >100 mm/hr 3
- Elevated C-reactive protein (CRP)
Other Laboratory Abnormalities
- Transient renal and hepatic function abnormalities may be present in adults 3
- Leukocytosis
Treatment of Rheumatic Fever
Eradication of Streptococcal Infection
First-line therapy: Full course of penicillin 2
- Penicillin V: 250 mg orally 2-3 times daily for 10 days, OR
- Benzathine penicillin G: 1.2 million units IM as a single dose
For penicillin-allergic patients:
- Narrow-spectrum cephalosporin (if non-anaphylactic allergy)
- Clindamycin: 20 mg/kg/day in 3 divided doses (max 1.8 g/day) for 10 days
- Azithromycin: 12 mg/kg once daily (max 500 mg) for 5 days 2
Anti-inflammatory Treatment
Aspirin: 75-100 mg/kg/day divided into 4-5 doses for 4-6 weeks 2
- Monitor for hepatotoxicity and gastric irritation
- Taper over 2-4 weeks after normalization of acute phase reactants
Corticosteroids: Consider in severe carditis, though evidence for improved long-term outcomes is limited 5
Secondary Prophylaxis
Intramuscular benzathine penicillin G: 1.2 million units every 4 weeks (every 3 weeks in high-risk situations) 1, 2
Alternative oral regimens:
Duration of Prophylaxis
- Rheumatic fever with carditis and residual heart disease: 10 years or until age 40, whichever is longer (sometimes lifelong) 1, 2
- Rheumatic fever with carditis but no residual heart disease: 10 years or until age 21, whichever is longer 1, 2
- Rheumatic fever without carditis: 5 years or until age 21, whichever is longer 1, 2
Monitoring and Follow-up
- Regular echocardiographic assessment to monitor valvular function 2
- Monitor acute phase reactants until normalized 2
- Early detection and treatment of streptococcal infections in family members 1
- Regular cardiac follow-up for patients with rheumatic heart disease 2
Prevention of Recurrence
- Strict adherence to prophylaxis regimen
- Endocarditis prophylaxis before high-risk procedures for patients with rheumatic heart disease 2, 6
- Prompt recognition and treatment of streptococcal pharyngitis in patients and family members 1
Pitfalls and Caveats
- Diagnosis is clinical with no laboratory gold standard 7
- Rheumatic fever can occur even after asymptomatic streptococcal infections 1
- Treatment can be safely initiated up to 9 days after symptom onset and still prevent acute rheumatic fever 2
- Recurrent attacks can lead to worsening of rheumatic heart disease 1
- Chorea may appear weeks to months after the initial infection, making the connection to streptococcal infection less obvious 4