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Condition/Disorder Synonym

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  • Acute rheumatic fever

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ICD-9-CM Codes

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  • 390 Rheumatic fever without mention of heart involvement

  • 391 Rheumatic fever with heart involvement

  • 392 Rheumatic chorea

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ICD-10-CM Codes

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  • I00 Rheumatic fever without heart involvement

  • I01.0 Acute rheumatic pericarditis

  • I01.1 Acute rheumatic endocarditis

  • I01.2 Acute rheumatic myocarditis

  • I01.8 Other acute rheumatic heart disease

  • I01.9 Acute rheumatic heart disease, unspecified

  • I02.0 Rheumatic chorea with heart involvement

  • I02.9 Rheumatic chorea without heart involvement

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Preferred Practice Patterns1

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Key Features

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Description

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  • Autoimmune inflammatory disease that can develop as a sequela of streptococcal infection, group A

  • Characterized by inflammatory lesions of the joints, heart, blood vessels, subcutaneous tissue, and central nervous system

  • Form of endocarditis

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Essentials of Diagnosis

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  • Rheumatic fever has variable manifestations; therefore, no specific diagnostic test exists for diagnosis

  • Usually occurs approximately 20 days after strep throat or scarlet fever

  • Diagnosis is based on the Jones criteria; major and minor criteria used to confirm diagnosis

  • Diagnosed if individual meets two major criteria or one major and one minor crieteria with previous strep infection

    • Major criteria

      • Carditis

      • Polyarthritis

      • Sydenham chorea

      • Erythema marginatum

      • Subcutaneous nodules

    • Minor criteria

      • Polyrthralgia

      • Previous rheumatic fever or rheumatic heart disease

      • Fever

      • Elevated erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) levels

      • Prolonged PR interval

  • Evidence of preceding group A streptococcal infection: positive throat culture or rapid antigen test result

  • Elevated or rising streptococcal antibody titer

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General Considerations

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  • The most significant complication is rheumatic heart disease, which usually occurs after repeated bouts of acute illness

    • It can present as valvular stenosis, most commonly involving the mitral valve

    • These patients are prone to infective endocarditis and stroke

  • Chorea can present months after the inciting infection

  • Some physicians monitor ESR and restart activity when it normalizes

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Demographics

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  • Rheumatic fever is predominantly a disease of developing countries and is concentrated in areas of deprivation and crowding.

  • The risk of developing rheumatic fever after an episode of streptococcal pharyngitis has been estimated at 0.3 to 3%.

  • Overall incidence in the United States is less than 1 per 100,000.

  • It is most common in 5- to 15-year-old children.

  • A genetic predisposition to rheumatic fever does exist.

  • The disease does not have a major racial or gender predisposition.

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Clinical Findings

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Signs and Symptoms

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  • Signs and symptoms result from inflammation in the heart, joints, skin, or central nervous system.

  • Sore throat: 35% to 60% of ...

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