Rheumatic Fever, Acute

Occurs 2-4 weeks following Group A Strep pharyngitis (NOT skin infections), most frequently in children 5-15 years old.

Clinically, it is best characterized via the JONES major criteria and minor criteria.

JONES CRITERIA

Major Criteria 

1.  Joints – Migratory Arthritis – usually earliest manifestation, affects several large joints in quick succession, lasting days to weeks
2. Pancarditis – inflammation can affect any part of the heart: myocarditis, epicarditis, pericarditis, endocarditis, valvulitis.  The most common early valvular manifestion is actually mitral regurgitation.  Can also commonly get conduction abnormalities.
3.  Nodules – Subcutaneous nodules that are firm and painless, resolve in several weeks
4.  Erythema Marginatum – an evanescent rash, classically with pale centers with rounded or “serpiginous” margins
5.  Syndenham chorea – neurological involvement, usually occurs later, often up to 8 months afterwards

Minor Criteria

1. Arthralgias, 2.  Fever,  3.  Elevated ESR/CRP,   4. Prolonged PR interval

2 major, or 1 major + 2 minor, in the presence of a history of recent GAS infection, makes the diagnosis.
Treatment of Acute Rheumatic Fever
1) Abxs for eradication of GAS carriage, regardless of presence of symptoms --> PCN PO x 10 days, or PCN IM x 1 dose
2) Aspirin for anti-inflammatory relief
3) Supportive care for other cardiac manifestations

After PCN course, patients should immediately be put on prophylactic antibiotics long-term, due to the very high risk of recurrence and further cardiac damage.  Length depends on the initial presentation:
- Carditis with residual heart disease --> PCN for at least 10 years or until 40, some say lifelong
- Carditis but no residual heart disease --> PCN for at least 10 years or until 21
- No carditis --> PCN for at least 5 years or until 21
Px PCN regimen involves q4week IM injections of PCN, vs PCN PO BID.

(Chanu Rhee MD, 10/19/10)