Diagnosis
Clinical diagnosis made on the basis of the Jones Criteria: Evidence of preceding group A streptococcal infection, the presence of two major manifestations or of one major and two minor manifestations indicates a high probability of acute rheumatic fever.
Major manifestations: Carditis (ECHO is more sensitive at finding pathology (PubMed)), Polyarthritis, Chorea, Erythema marginatum, Subcutaneous nodules.
Minor manifestations: arthralgia, fever, elevated erythrocyte sedimentation rate or C-reactive protein, prolonged PR interval, evidence of antecedent Group A streptococcus infection, positive throat culture or rapid streptococcal antigen test, elevated or rising streptococcal antibody titer.
Epidemiologic Risks
Post-infectious from S. pyogenes.Microbiology
Due to infection with strains of Group A streptococcus i.e. S. pyogenes. Of interest,ß the strains in Hawaii are different than the strains in the mainland US and preferentially affect Samoans (PubMed).
Empiric Therapy
Bed rest if carditis.
- Arthralgia or mild arthritis; no carditis: analgesics.
- Moderate or severe arthritis; no carditis, or carditis with or without cardiomegaly, but without failure: Aspirin 90-100 mg/kg/day for 2 weeks; increased if necessary; 60-70 mg/kg/day for the subsequent 6 weeks.
- Carditis with failure, with or without joint manifestations: Prednisone 40-60 mg/day, after 2-3 weeks, taper slowly over 3 more weeks. Continue aspirin for a month after stopping of prednisone.
Prevention: Benzathine penicillin G 1,200,000 U IM every 4 wks OR penicillin V 250 mg po bid OR sulfadiazine 0.5 g po once daily 27 kg (60 lb); 1.0 g po once daily >27 kg (60 lb).
Allergy to penicillin and sulfadiazine: erythromycin 250 mg po bid.
Duration of prophylaxis. If rheumatic fever with carditis and residual heart disease (persistent valvular disease): At least 10 yr since the last episode and at least until age 40 yr, sometimes lifelong prophylaxis.
Rheumatic fever with carditis but no residual heart disease (no valvular disease): 10 yr or well into adulthood, whichever is longer.
Rheumatic fever without carditis: 5 yr or until age 21 yr, whichever is longer.
Pearls
In most of the world prior rheumatic heart disease a major cause of endocarditis.
Curious Cases
Relevant links to my Medscape blog
Last Update: 01/14/19.