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Snapshot
  • A 10-year-old girl presents with a fever and migrating joint pains in the knees and elbows. She reports having had a sore throat a few days ago. On physical exam, she is febrile and has raised erythematous serpentine-like lesions on her back. A rapid streptococcal test is positive and there is an elevated erythrocyte sedimentation rate. She is given antibiotics for this disease.
Introduction
  • Clinical definition
    • acute inflammation of multiple systems caused by an immunologic reaction to group A streptococcus infection
  • Epidemiology
    • demographics
      • children and adolescents
      • most common in developing nations
    • location
      • mitral valve > aortic valve > tricuspid valve
      • most commonly affects the high-pressure valves
    • risk factors
      • poverty and overcrowding
      • preceding group A streptococcal pharyngitis
  • Etiology
    • typically due to inadequate treatment of group A streptococcal infection
  • Pathogenesis
    • abnormal immune response to group A streptococci
      • molecular mimicry between streptococcal M protein and self-proteins including cardiac proteins, keratin, laminin, and vimentin
      • immune-mediated (type II) hypersensitivity
    • disease is characterized by
      • carditis
      • arthritis
      • Sydenham chorea
        • caused by antibodies that cross the blood-brain barrier and bind dopamine D1 and D2 receptors
      • erythema marginatum
      • subcutaneous nodules
  • Associated conditions
    • Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infection (PANDAS)
  • Prognosis
    • occurs 2-3 weeks after pharyngitis, except carditis and chorea
      • carditis occurs after months
      • chorea presents after months
    • risk of recurrence decreases with time
Presentation
  • Symptoms 
    • migratory joint pains, especially in the knees, ankles, and elbows
    • chest discomfort
    • heart failures (rare)
  • Physical exam
    • fever
    • may have dyspnea
    • new murmur on cardiac exam may indicate carditis
    • firm and painless subcutaneous nodules over bony prominences
      • often occurs with carditis
    • erythema marginatum
      • evanescent raised ring-like or serpentine lesion on trunk
    • Sydenham chorea
      • involuntary, jerky, purposeless movements of the hands, feet, face, or tongue
Imaging
  • Echocardiography
    • indications
      • when murmur is auscultated on examination suspicious for rheumatic heart disease
      • to confirm complication of acute rheumatic fever
    • findings
      • valvular abnormalities, including regurgitations or stenosis
Studies
  • Positive throat culture or rapid antigen test for Streptococcus
  • Labs
    • ↑ anti-streptolysin O (ASO) titers
    • ↑ anti-deoxyribonuclease B titers
  • Electrocardiography
    • prolonged PR interval
      • first-degree heart block
  • Histology
    • Aschoff bodies
      • granuloma with giant cells on heart valves
    • Anitschkow cells
      • enlarged macrophages within Aschoff bodies
  • Making the diagnosis
    • based on clinical presentation, clinical or laboratory evidence of preceding group A streptococcal infection, and confirmation with 2 major Jones criteria or 1 major and 1 minor
 
Jones Criteria
Major Criteria
Minor Criteria
  • Joints (migratory arthritis)
  • O looks like the heart (carditis)
  • Nodules (subcutaneous)
  • Erythema marginatum
  • Sydenham chorea
  • Fever
  • Antecedent strep infection
  • Arthralgias
  • ↑ Erythrocyte sedimentation rate
  • ↑ C-reactive protein
  • First-degree heart block
 
Differential
  • Infective endocarditis
    • distinguishing factors
      • no association with group A streptococcal infection
      • other findings including Roth spots, Osler nodes, Janeway lesions, and splinter hemorrhages on nail bed
      • vegetations seen on valves on imaging
Treatment
  • Management approach
    • patients must be treated adequately with antibiotics to prevent rheumatic heart disease
    • patients must then be put on long-term prophylaxis to prevent recurrence
  • Medical
    • penicillins
      • indication
        • for all patients
    • macrolides
      • indications
        • for patients allergic to penicillin
    • nonsteroidal anti-inflammatory drugs (NSAIDs)
      • indication
        • for patients with joint pain or fever
Complications
  • Rheumatic heart disease
 

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