Updated: 6/18/2021

Acute Rheumatic Fever

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  • summary
    • Acute Rheumatic Fever is a systemic reaction following Streptococcal pharyngitis which causes a characteristic rash and migratory arthritis that involves multiple large joints.
    • Diagnosis is based on the Revised Jones criteria.
    • Treatment is usually oral antibiotics and anti-inflammatories.
  • Epidemiology
    • Incidence
      • 500k new cases per year worldwide
    • Demographics
      • children 5-15 years of age
      • lower socioeconomic status
      • developing countries
  • Etiology
    • Pathophysiology
      • molecular biology
        • autoimmune reaction within 1-4 weeks following Group A beta-hemolytic Streptococcus pyogenes pharyngitis
        • molecular mimicry
          • antibodies to M protein of GAS causing an inflammatory response to synovium, myocardium, and basal ganglia cells
  • Presentation
    • Symptoms
      • rash
      • joint pain and refusal to bear weight
    • Physical exam
      • arthritis
        • red, swollen joints predominantly if the lower extremities, migratory
      • rash
        • erythema marginatum (pink rash on trunk and extremities that spares the face)
      • other
        • subcutaneous nodules on upper extremity extensor surfaces
        • chorea
        • carditis, first-degree heart block (prolonged PR interval)
  • Imaging
    • Radiographs
      • may show effusion but often unremarkable
    • Ultrasound/MRI
      • equivocal cases where concern remains for septic arthritis
    • Echocardiogram
  • Lab Studies
    • Serum labs
      • antistreptolysin O titers elevated in 80%
      • peripheral blood leukocytosis
      • inflammatory markers
        • ESR elevated
        • CRP elevated
    • Synovial Fluid Analysis
    • Synovial Fluid Analysis
      Condition
      WBC (cells/mL)
      PMNs (%)
      Normal
      < 200
      < 25%
      Effusion from trauma
      < 5,000
      < 25%
      Toxic synovitis
      5,000-15,000
      < 25%
      Acute rheumatic fever
      10,000-15,000
      50%
      JRA
      15,000-80,000
      75%
      Septic arthritis
      > 50,000
      > 75%
  • Differential
    • Lyme disease
      • positive Lyme titers
    • Septic arthritis
      • purulent synovial fluid, elevated cell count
    • Reiter/Reactive arthritis
      • specific rheumatologic labs will be positive (HLA-B27)
    • Juvenile Rheumatoid Arthritis
      • specific rheumatologic labs will be positive (RF and ANA)
    • Gout/Pseudogout
      • synovial fluid positive for MSU or CPPD crystals
  • Diagnosis
    • Diagnosis based on Revised Jones criteria
      • preceding Strep infection with 2 major criteria or 1 major and 2 minor criteria
        • major criteria
          • carditis
          • polyarthralgia
          • chorea
          • erythema marginatum
          • subcutaneous nodules
        • minor criteria
          • fever
          • arthralgia
          • prior rheumatic fever
          • increased ESR
          • prolonged PR interval (First degree heart block)
          • prior Group A Strep infection
  • Treatment
    • Nonoperative
      • oral antibiotics and anti-inflammatories
        • indications
          • acute infection confirmed by Modified Jones criteria
          • arthralgias and/or fever
        • modalities
          • antibiotics
            • Penicillin 250mg PO BID x10 days (or erythromycin if penicillin-allergic)
            • Penicillin monthly x10 years to prevent rheumatic fever recurrence
          • anti-inflammatories
            • naprosyn more commonly used
            • salicylates used historically but concern for liver derangement deters use
  • Complications
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