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Key Features

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Essentials of Diagnosis
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  • Except in cases of rheumatic fever manifesting solely as Sydenham chorea or long-standing carditis, there should be clear evidence of a streptococcal infection such as

    • Scarlet fever

    • Positive throat culture for group A β-hemolytic streptococcus

    • Increased antistreptolysin O or other streptococcal antibody titers

  • The antistreptolysin O titer is significantly higher in rheumatic fever than in uncomplicated streptococcal infections

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General Considerations
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  • Overall incidence in the United States is < 1 per 100,000

  • Peak age of risk in the United States is 5–15 years

  • Disease is slightly more common in girls and in African Americans

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Clinical Findings

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  • Carditis

    • Implies pancardiac inflammation, but it may be limited to valves, myocardium, or pericardium

    • Valvulitis is frequently seen, with the mitral valve most commonly affected

    • Mitral insufficiency is the most common valvular residua

    • An early decrescendo diastolic murmur consistent with aortic insufficiency is occasionally encountered as the sole valvular manifestation

    • Most serious consequence of rheumatic fever

    • Varies from minimal to life-threatening heart failure (HF)

  • Polyarthritis

    • The large joints (knees, hips, wrists, elbows, and shoulders) are most commonly involved

    • Arthritis is typically migratory

    • Joint swelling and associated limitation of movement should be present

    • Occurs in 80% of patients

  • Sydenham chorea

    • Characterized by involuntary and purposeless movements

    • Often associated with emotional lability

    • Symptoms become progressively worse and may be accompanied by ataxia and slurring of speech

    • Muscular weakness becomes apparent following the onset of the involuntary movements

    • Chorea is self-limiting, although it may last up to 3 months

    • Chorea may not be apparent for months to years after the acute episode of rheumatic fever

  • Erythema marginatum

    • A macular, serpiginous, erythematous rash with a sharply demarcated border appears primarily on the trunk and the extremities

    • Face is usually spared

  • Subcutaneous nodules

    • Usually occur only in severe cases, and then most commonly over the joints, scalp, and spinal column

    • Nodules vary from a few millimeters to 2 cm in diameter and are nontender and freely movable under the skin

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Diagnosis

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Jones criteria (modified)
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  • Major manifestations

    • Carditis

    • Polyarthritis

    • Sydenham chorea

    • Erythema marginatum

    • Subcutaneous nodules

  • Minor manifestations: Clinical

    • Previous rheumatic fever or rheumatic heart disease

    • Polyarthralgia

    • Fever

  • Minor manifestations: Laboratory

    • Acute phase reaction: elevated erythrocyte sedimentation rate, C-reactive protein, leukocytosis

    • Prolonged PR interval plus

    • Supporting evidence of preceding streptococcal infection, ie, increased titers of antistreptolysin O or other streptococcal antibodies, positive throat culture for group A streptococcus

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Treatment

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  • Long-acting benzathine penicillin

    • Drug of choice

    • Depending on the age and weight of the patient, a single intramuscular injection of 0.6–1.2 million units is effective

  • Alternative penicillin regimens include

    • Penicillin V, 250–500 mg orally 2–3 times a day for 10 days or

    • Amoxicillin, 50 mg/kg (maximum 1 g) once daily for 10 days

  • For patients allergic to penicillin,

    • Narrow-spectrum cephalosporins

    • Clindamycin

    • Azithromycin

    • Clarithromycin

  • Aspirin, 30–60 mg/kg/d in four divided ...

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