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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Carditis in almost 50% of those with acute rheumatic fever: heart failure, new regurgitant heart murmur (mitral or aortic regurgitation), pericarditis

  • Evidence of streptococcal infection: throat culture, detection of streptococcal antibodies

  • Chorea

  • Polyarthritis

  • Typical skin findings: subcutaneous nodules, erythema marginatum

GENERAL CONSIDERATIONS

  • Rheumatic fever is the most common cause of cardiac disease worldwide in children and young adults

  • The disease is more common in developing countries (100 per 100,000) compared with developed countries (2 per 100,000)

  • The rheumatogenic strains of Streptococcus have M proteins that share epitopes with cardiac myosin and sarcolemmal membrane proteins

    • – Host antibodies against these epitopes cross-react with cardiac tissue, producing an inflammatory disease of all 3 layers of the heart

  • During an outbreak of group A streptococcal pharyngitis, up to 5% get rheumatic fever

  • The characteristic lesion in cardiac tissue samples is composed of multinucleated giant cells and is called an Aschoff nodule

    • – These nodules are seen only in cardiac tissue despite widespread inflammation in other tissues

  • Long-term sequelae of rheumatic fever occur only in the heart despite acute involvement of other tissue

  • The mitral valve is most commonly affected, followed by aortic, tricuspid, and pulmonic valves

  • The valves are thickened from inflammation, and the annuli are dilated, producing regurgitation (the most common finding)

  • Pericarditis is frequent, but tamponade is rare

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Major manifestations:

    • – Polyarthritis

    • – Chorea

    • – Subcutaneous nodules

    • – Erythema margination

    • – Carditis (eg, new valve regurgitation murmurs)

  • Minor manifestations:

    • – Arthralgia

    • – Fever

    • – Elevated acute-phase reactants (eg, erythrocyte sedimentation rate [ESR] and C-reactive protein)

    • – Prolonged PR interval on ECG

  • Supporting evidence of antecedent group A streptococcal infection:

    • – Positive throat culture

    • – Positive rapid streptococcal antibody test

    • – Elevated or rising streptococcal antibody titer

  • The modified Jones Criteria for the diagnosis of rheumatic fever require 2 major criteria or 1 major and 2 minor criteria with supporting evidence of streptococcal infection

PHYSICAL EXAM FINDINGS

  • Carditis:

    • – Apical pansystolic murmur of mitral regurgitation

    • – Apical mid-diastolic murmur (Carey Coombs murmur)

    • – Basal diastolic decrescendo murmur of aortic regurgitation (50%)

  • Polyarthritis: 2 or more large joints (knees, ankles, elbows, wrists) showing redness, swelling, and heat in a migratory pattern for 1 day to 3 weeks

  • Chorea (a late manifestation [3 or more months after infection]): involuntary movements while awake

  • Erythema margination: pink macular nonpruritic rash on the trunk or extremities (never the face), which appear as rings that may coalesce and look serpiginous (10%)

  • Subcutaneous nodules: discrete firm painless mobile nodules (0.5–2 cm) on the extensor surfaces of joints or bony prominences, which occur about 3 weeks after other signs

DIFFERENTIAL DIAGNOSIS

  • Juvenile rheumatic arthritis

  • Infective endocarditis

  • Myocarditis due to other causes

  • Pericarditis due to other causes

  • Mitral ...

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