Acute Rheumatic Fever

Aka: Acute Rheumatic Fever, Rheumatic Fever, Rheumatic Heart Disease, Erythema Marginatum, Jones Criteria, Modified Jones Criteria

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II. Epidemiology

  1. Incidence (per year in school aged children)
    1. U.S.: <2 cases per 100,000
    2. Worldwide: 150 cases per 100,000 (2.8 Million new cases/year)
  2. Prevalence (esp. developing world)
    1. U.S.: 714,000 (in 2021)
    2. Worldwide: 40-55 Million
  3. Mortality
    1. Worldwide Mortality: >230,000 attributed deaths/year
  4. Attack rate: 0.3 to 3% of untreated Streptococcal Pharyngitis cases
  5. Peak age of onset: 6 to 12 years old (rare under age 3 years)

III. Pathophysiology

  1. Follows Streptococcal Pharyngitis by 2-6 weeks (typically 10-28 days)
  2. Autoimmune reaction to Group A Stretococcal Infection
    1. Streptococcal Pharyngitis
    2. Scarlet Fever
    3. Impetigo is NOT associated with Rheumatic Fever
  3. Autoimmune reaction results in focal tissue Inflammation
    1. Heart (esp. mitral valve, aortic valve)
    2. Nervous system (Sydenham's Chorea)
    3. Skin (Subcutaneous Nodules, Erythema Marginatum)
    4. Joints (Migratory Arthritis, starting with knees and ankles)

IV. Risk Factors

  1. Low income, health disparities, developing world (predominance of cases)
  2. Genetic predisposition

V. Diagnosis: Jones Criteria

  1. Diagnostic criteria
    1. Two Major Criteria or
    2. One Major and 2 Minor Criteria
  2. Major Criteria
    1. Mnemonic: Jones
      1. J - Joints (Migratory Arthritis)
      2. O - Shape of Heart (Carditis)
      3. N - Nodules (Subcutaneous)
      4. E - Erythema Marginatum
      5. S - Sydenham's Chorea
    2. Carditis
      1. Present in 50-60% of cases
      2. Findings
        1. New Heart Murmur (Mitral Regurgitation)
        2. New Heart Failure (Dyspnea, Orthopnea, edema)
        3. Electrocardiogram abnormalities (e.g. Tachycardia, Heart Block)
        4. Echocardiogram abnormalities (valvular Nodules or other valve abnormalities)
      3. Conditions
        1. Pericarditis
        2. Myocarditis (rare)
        3. Endocarditis (mitral valve most commonly affected)
          1. Results in Rheumatic Heart Disease
          2. Manifests as valve disease in 10-20 years after carditis in 50%
          3. Mitral Stenosis is most common outcome
    3. Migratory polyarthritis
      1. Monoarthritis meets major criteria IF patient is in a moderate to high risk population group
        1. Monoarthritis meets only minor criteria for low risk patients (AHA 2015 guidelines)
        2. Polyarthritis (NOT polyarthralgia) meets major criteria regardless of risk grouping
      2. Most common symptom (occurs in 49-78% of patients)
      3. Significant response to Aspirin or NSAIDS
      4. Migratory Arthritis
      5. Large joints starting with legs and moving to arms
        1. Knees and ankles
        2. Wrists and elbows
        3. Rarely affects spine
      6. Transient (3 days per joint, and 3 weeks total)
        1. Typical course is <1 week
      7. More severe in teens and young adults
    4. Sydenham's Chorea
      1. Abrupt onset of choreoform movements (purposeless, nonrhythmic, involuntary) only present while awake
      2. Motor weakness
      3. Emotional disturbance (outbursts, Psychosis)
      4. Late finding (may occur up to 8 months after initial infection)
      5. Resolves over 2-3 years
    5. Erythema Marginatum (associated with carditis)
      1. Non-pruritic pink to slightly red rings
      2. Involves trunk and proximal extremities, but spares face
      3. Transiently appears, rapidly advances and disappears over months
    6. Subcutaneous Nodules (associated with carditis)
      1. Small, firm painless Nodules on extensor surfaces (esp. dorsal wrist, elbow, anterior knee)
      2. Develop over bony prominences or over tendons
  3. Minor Criteria
    1. Polyarthralgias
    2. Fever >101.3 F (>38.5 C)
    3. Prolonged PR Interval on Electrocardiogram
    4. Elevated acute phase reactants
      1. Elevated Sedimentation Rate (ESR) >=60 mm/h (>=30 mm/h if moderate-high risk patient)
      2. Elevated C-Reactive Protein >=3 mg/dl
  4. Supporting criteria
    1. Group A Streptococcal Infection precedes episode
      1. Positive GAS Rapid Antigen test or Throat Culture OR
      2. Anti-streptococcal Antibody level increased

VI. Differential Diagnosis: Migratory polyarthritis with cardiac involvement, rash

  1. Myocarditis
  2. Lyme Disease (Lyme Carditis)
  3. Systemic Lupus Erythematosus
  4. Bacterial Endocarditis
  5. Juvenile Idiopathic Arthritis (not associated with cardiac involvement)

VII. Labs

  1. Complete Blood Count
    1. Variable Leukocytosis
  2. Acute phase reactants markedly increased
    1. Erythrocyte Sedimentation Rate (ESR)
    2. C-Reactive Protein (CRP)
  3. Group A Beta Hemolytic Streptococcus testing
    1. Rapid Antigen test with reflex to Throat Culture
    2. ASO Titer
      1. Increased over baseline

VIII. Diagnostics

  1. Electrocardiogram
    1. Prolonged PR Interval (AV Block)
    2. Pericarditis
      1. See Electrocardiogram in Pericarditis

IX. Imaging

  1. Chest XRay
    1. Signs of Congestive Heart Failure
  2. Echocardiogram
    1. Mitral Insufficiency (Mitral Regurgitation)
    2. Aortic Insufficiency (Aortic Regurgitation)

X. Course

  1. Onset 10-28 days after Streptococcal Pharyngitis onset

XI. Management

  1. Admit for definitive diagnosis and evaluation
  2. Polyarthritis
    1. Aspirin
      1. Adult: 4-8 g/day
      2. Child: 80-100 mg/kg/day
        1. Consider Naproxen instead due to risk of Reye's Syndrome
    2. NSAIDs
      1. Consider if Aspirin cannot be used
  3. Carditis
    1. Corticosteroids as directed by cardiology
    2. Congestive Heart Failure management (e.g. Furosemide, ACE Inhibitor)
    3. Atrial Fibrillation management (e.g. Atrial Fibrillation Rate Control with Beta Blockers)
  4. Treat Streptococcal Pharyngitis in all cases
    1. See Streptococcal Pharyngitis for treatment regimens
    2. Penicillin G Benzathine (Bicillin LA) IM is typically used
      1. Oral Erythromycin may be used if Penicillin allergic
  5. Prevention of recurrence
    1. Rheumatic Fever with carditis and residual heart disease (valvcular disease persists on echo or exam)
      1. Benzathine Penicillin G IM monthly for 10 years or until age 40 years (whichever is longer)
    2. Rheumatic Fever with carditis but NO residual heart disease
      1. Benzathine Penicillin G IM monthly for 10 years or until age 21 years (whichever is longer)
    3. Rheumatic Fever without carditis
      1. Benzathine Penicillin G IM monthly for 5 years or until age 21 years (whichever is longer)
    4. References
      1. Gerber (2009) Circulation 119(11): 1541-51 [PubMed]

XII. Prevention

  1. Antibiotics for Streptococcal Pharyngitis
  2. Test and treat household contacts of patients with Streptococcal Pharyngitis
    1. May empirically treat symptomatic close, household contacts

XIII. References

  1. Carvey (2025) Crit Dec Emerg Med 39(12): 4-12
  2. Carapetis (2005) Lancet 366(9480): 155-68 [PubMed]
  3. Madden (2009) Canadian Family Physician 55: 475-8 [PubMed]
  4. Maness (2018) Am Fam Physician 97(8): 517-22 [PubMed]

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