Cardiovascular Medicine Book

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Acute Rheumatic Fever

Aka: Acute Rheumatic Fever, Rheumatic Fever, Rheumatic Heart Disease, Erythema Marginatum, Jones Criteria, Modified Jones Criteria
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  1. Epidemiology
    1. Incidence: 20 million cases/year in developing world
  2. Pathophysiology
    1. Autoimmune reaction to Group A Stretococcal Infection
      1. Streptococcal Pharyngitis
      2. Scarlet Fever
      3. Impetigo never results in Rheumatic Fever
  3. Diagnosis: Jones Criteria
    1. Diagnostic criteria
      1. Two Major Criteria or
      2. One Major and 2 Minor Criteria
    2. Major Criteria
      1. Carditis
        1. Endocarditis (mitral valve most commonly affected)
          1. Results in Rheumatic Heart Disease
          2. Manifests in 10-20 years after carditis in 50%
          3. Mitral Stenosis is most common outcome
        2. Pericarditis
        3. Myocarditis (rare)
      2. Polyarthritis
        1. Most common symptom (occurs in 49-78% of patients)
        2. Migratory arthritis
        3. Large joints starting with legs and moving to arms
          1. Knees and ankles
          2. Wrists and elbows
          3. Rarely affects spine
        4. Transient (3 days per joint, and 3 weeks total)
          1. Typical course is <1 week
        5. More severe in teens and young adults
      3. Sydenham's Chorea
        1. Late finding (may occur years later)
      4. Erythema Marginatum
        1. Non-pruritic erythematous rings on trunk
        2. Transiently appear and disappear over months
      5. Subcutaneous Nodules
        1. Small painless Nodules on extensor surfaces
    3. Minor Criteria
      1. Arthralgias
      2. Fever
      3. Elevated Sedimentation Rate (ESR)
      4. Elevated C-Reactive Protein
      5. Prolonged PR Interval on Electrocardiogram
    4. Supporting criteria
      1. Group A Streptococcal Infection precedes episode
  4. Labs
    1. ASO Titer increased
  5. Course
    1. Onset 2-3 weeks after Streptococcal Pharyngitis onset
  6. Prevention
    1. Antibiotics for Streptococcal Pharyngitis

Erythema marginatum (C0085659)

Definition (AIR) WHAT: Erythema marginatum. Erythema Marginatum: an evanescent, erythematous rash characteris- tically (though not commonly) seen in the early stages of rheumatic fever. The rash is not limited to rheumatic fever, however, and has also been reported in patients with allergic drug reactions, sepsis and glomerulonephritis. WHY: Although the rash is not pathognomonic of rheumatic fever, its presence is helpful in conjunction with other manifestations of rheumatic fever, particularly carditis with which it is most often associated. HOW: The rash is characterized by pink, evanescent, slightly raised small macules with a sharply demarcated and irregular border. The erythematous areas often have pale centers. The rash commonly occurs over the trunk and inner aspects of the upper arms and thighs, but never on the face. It is non-painful and rarely pruritic, may appear and disappear in a matter of hours only to return, and may be brought out by a hot bath or shower. The rash blanches on pressure, and is a transient rash which tends to migrate from place to place. REFS: 1) Jones criteria (revised) for guidance in the diagnosis of rheumatic fever. Circulation 32:664, 1965. 2) Dictionary of the Rheumatic Diseases, Volume I: Signs and Symptoms. American Rheumatism Association, 1982. (Descriptor #133).
Concepts Disease or Syndrome (T047)
ICD10 L53.2
SnomedCT 238817005, 50610002
English ERYTHEMA MARGINATUM, erythema marginatum, erythema marginatum (diagnosis), Erythema annulare rheumaticum (disorder), Erythema marginatum, Erythema annulare rheumaticum, Erythema marginatum (disorder), erythema; marginatum, marginatum; erythema
Italian Eritema marginato, Eritema anulare reumatico
Dutch erythema anulare rheumaticum, erytheem; marginatum, marginatum; erytheem, Erythema marginatum, erythema marginatum
French Erythème annulaire rhumatismal, Erythème marginé
German Erythema annulare rheumaticum, Erythema marginatum
Portuguese Eritema anular reumático, Eritema marginado
Spanish Eritema anular reumático, Erythema annulare rheumaticum, eritema anular reumático, eritema marginado (trastorno), eritema marginado, Eritema marginado
Japanese 辺縁紅斑, リウマチ性環状紅斑, ﾍﾝｴﾝｺｳﾊﾝ, ﾘｳﾏﾁｾｲｶﾝｼﾞｮｳｺｳﾊﾝ
Czech Erythema annulare rheumaticum, Erythema marginatum
Korean 유연성 홍반
Hungarian Erythema annulare rheumaticum, Erythema marginatum
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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