Infectious Disease Compendium

Rheumatic Fever

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 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.

Pearls

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

Rants

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.