Infectious Disease Compendium

Rheumatic Fever


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.


Due to infection with strains of Group A streptococcus i.e. S. pyogenes. Of interest the strains in Hawaii are different than the strains in the mainland US and preferentially affects Samoan (1).

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 Rheumatic fever with carditis and residual heart disease (persistent valvular disease): At least 10 yr since 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.


In most of the world prior rheumatic heart disease a major cause of endocarditis.


ICD9 Codes (Soon to be supplanted by ICD10)

rheumatic fever 390; with heart involvement 391.9; carditis 391.9; endocarditis (aortic) (mitral) (pulmonary) (tricuspid) 391.1; multiple sites 391.8; myocarditis 391.2; pancarditis, acute 391.8; pericarditis 391.0; specified type NEC 391.8; valvulitis 391.1; inactive or quiescent with; cardiac hypertrophy 398.99; carditis 398.90; endocarditis 397.9; aortic (valve) 395.9; with mitral (valve) disease 396.9; mitral (valve) 394.9; with aortic (valve) disease 396.9; pulmonary (valve) 397.1; tricuspid (valve) 397.0; heart conditions (classifiable to 429.3, 429.6, 429.9) 398.99; failure (congestive) (conditions classifiable to 428.0, 428.9) 398.91; left ventricular failure (conditions classifiable to 428.1) 398.91; myocardial degeneration (conditions classifiable to 429.1) 398.0; myocarditis (conditions classifiable to 429.0) 398.0; pancarditis 398.99; pericarditis 393.