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Updated: Jul 31 2022

Rheumatic Fever

Images marginatum.jpg,_rheumatic_myocarditis(1).jpg
  • 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 barely 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)
        • believed to be an autoimmune condition caused by antibodies targeting the basal ganglia after group A streptococcal infection
        • resulting in acute onset of obsessive compulsive disorder and/or tics
  • 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 flat or barely 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
  • 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
    • 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
  • 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
  • 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
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