rheumatic fever

Introduction

An inflammatory condition that occasionally follows group A streptococcal pharyngitis. It tends to recur.

Etiology

• group A streptococcus, generally pharyngitis

Epidemiology

• affects mostly children 5-15 years of age,
• may also occur in adults, especially in crowded conditions

Pathology

• subset of Streptococcal M proteins implicated
• strains that predispose to rheumatic fever & those that predispose to poststreptococcal glomerulonephritis are mutually exclusive

Clinical manifestations

• arthritis*
  • involvement of several joints*, knees most commonly
  • exquisitely painful
  • classically migratory, but generally not
  • generally follows pharyngitis by 2-3 weeks, but 1/3 of cases occur in the absence of antecedent pharyngitis
• carditis*
  • murmur
  • mitral valve most commonly involved
  • rare in adults
• chorea* (uncommon)
• erythema marginatum*
• subcutaneous nodules*
• fever is minor criteria

* Jones criteria, major manifestations;

diagnosis by Jones criteria: 2 major or 1 major & 2 minor criteria

Laboratory

• elevated antistreptolysin O (ASO) titer
• elevated erythrocyte sedimentation rate >= 60 mm/hr*
• elevated C-reactive protein >= 3 mg/dL
• Streptozyme assay
• blood cultures to rule out endocarditis
• throat culture for Streptococcus

* minor criteria

Diagnostic procedures

• EKG (PR prolongation*)
• echocardiogram (all patients)
• arthrocentesis of affected joints

* minor criteria

Radiology

• chest X-ray to rule out cardiomegaly

Management

• benzathine penicillin G IM once vs penicillin V or amoxicillin for 10 days
• acute rheumatic fever
  • non-steroidal anti-inflammatory agents (NSAIDs)
    • aspirin 80 mg/kg/day for 1st 2 weeks, then 60 mg/kg/day for 6 weeks
  • prednisone 40-60 mg QD
    • useful for controlling pericarditis & CHF
    • no effect on residual heart disease
• prophylaxis (documented rheumatic fever - recommendations may be outdated)
  • long-term prophylactic penicillin for at least 10 years after last episode
    • until at least age 40 if valvular heart disease
    • until at least age 21 if no valvular heart disease
  • benzathine penicillin G 1.2 million units IM every 4 weeks
  • erythromycin 100-250 mg PO BID
  • sulfadiazine 0.5-1 g PO QD
  • erythromycin 100-250 mg PO BID
  • prophylaxis for bacterial endocarditis prior to surgical procedures

More general terms

• autoimmune disease
• infectious complication; infectious sequella

More specific terms

• rheumatic heart disease

Additional terms

• Jones criteria for rheumatic fever

Patient information

rheumatic fever patient information