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Rheumatic Fever

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Topic updated on 09/11/17 10:08am

Snapshot
  • PhotoA 13-year-old-boy presents with 3 days of malaise, painful joints, nodular swelling over his elbows, and a low-grade fever. Two weeks ago he was seen by his pediatrician for a sore throat. You observe a well-defined rash on his trunk and shoulders.
Introduction
  • An autoimmune disease sequela of untreated pharyngeal streptococcal infection
  • Caused by cross-reactions between strep antigens and antigens on joint and heart tissue
  • Systemic immune process that may or may not lead to RHD
  • Risks include right-sided heart disease
    • secondary to valvular dysfunction
    • most often mitral stenosis
Presentation
  • Symptoms 
    • usually present approximately two weeks following Streptococcal pharyngitis
    • joint pain
    • rash
    • skin nodules
    • neurlogic dysfunction (motor instability)
    • fever
  • Physical exam
    • diagnoseusing the Jones criteria
      • evidence of a preceding group A streptococcal infection plus
        • two major critertia or
        • one major and two minor criteria

      Major criteria (PECCS)
      Minor criteria
      • Joints - arthritis
      • <3 Carditis (myocarditis, pericarditis)
      • Nodules - subcutaneous
      • Erythema marginatum
      • Syndenham's chorea
      • Fever
      • Antecedent strep infection
      • Athralgias
      • Acute phase reactions
        • elevated ESR and/or CRP
      • Prolonged PR interval
Evaluation
  • Diagnosis is based primarily on clinical observation (Jones' criteria) and history
  • Labs
    • positive ASO (reflects past immunologic event)
    • elevated ESR and/or CRP
    • Rapid antigen detection test (RADT) +/- throat culture
  • EKG
    • may show evidence of RHD secondary to mitral valve involvement
  • Echo
Differential
  • RA, SLE, endocarditis, osteomyelitis, Lyme's Disease, sickle cell disease
Treatment
  • Medical management
    • amoxicillin
    • penicillin
      • indicated for acute rhuematic fever
    • azithromycin in penicillin-allergic patients
Prognosis, Prevention, and Complications
  • Prognosis
    • good to very good if diagnosed and treated aggressively
    • antibiotic prophylaxis is key to maintaining good outcomes
  • Prevention
    • primary prophylaxis: prompt treatment of GAS infection
    • secondary prophylaxis: after rheumatic fever (if no carditis) until age of 21 or 5 years to reduce recurrent episodes and subsequent complications
  • Complications
    • RHD secondary to autoimmune response affecting mitral valve


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