Skip to Main Content

For further information, see CMDT Part 10-48: Rheumatic Fever

Key Features

Essentials of Diagnosis

  • More common (100 cases/100,000 population) in developing countries than in the United States (approximately 2 cases/100,000 population)

  • Peak incidence ages 5–15 years

  • Revision of Jones criteria in 2015 includes echocardiographic findings

  • May involve mitral and other valves acutely, rarely leading to heart failure

General Considerations

  • Rheumatic fever

    • A systemic immune process that is a sequela of a β-hemolytic streptococcal infection of the pharynx

    • Pyodermic infections are not associated with rheumatic fever

    • Rheumatic carditis and valvulitis

      • May be self-limited

      • May lead to slowly progressive valvular deformity

      • A perivascular granulomatous reaction with valvulitis is characteristic lesion

  • Chronic rheumatic heart disease

    • Results from single or repeated attacks of rheumatic fever that produce

      • Rigidity and deformity of valve cusps

      • Fusion of the commissures

      • Shortening and fusion of the chordae tendineae

    • Valvular stenosis or regurgitation results and the two often coexist

    • Affects mitral, aortic, and tricuspid valves, but the pulmonary valve only very rarely


  • Rheumatic fever

    • Rare before age 4 or after age 40 years

    • Responsible for 320,000 deaths in young people worldwide each year

    • Over 15 million have evidence for rheumatic heart disease

  • Rheumatic heart disease remains a major cardiovascular problem in the poorest regions of the world

Clinical Findings

Symptoms and Signs

  • Signs of acute rheumatic fever

    • Usually commence 2–3 weeks after infection

    • May appear as early as 1 week or as late as 5 weeks

Major Criteria

  • Carditis

    • Pericarditis

    • Cardiomegaly

    • Heart failure, with painful liver engorgement due to tricuspid regurgitation

    • Mitral or aortic regurgitation murmurs

  • Carey–Coombs short mid-diastolic mitral murmur may be present due to inflammation of the mitral valve

    • Occurs most often in children and adolescents

    • When any of the above definitive signs are absent, the diagnosis of carditis depends on the following less specific abnormalities:

      • ECG changes, including changing contour of P waves or inversion of T waves

      • Changing quality of heart sounds

      • Sinus tachycardia, arrhythmia, or ectopic beats

  • Erythema marginatum

    • Begins as rapidly enlarging macules that assume the shape of rings or crescents with clear centers; may be less notable on Black skin

    • May be raised, confluent, and either transient or persistent

  • Subcutaneous nodules

    • Small (≤ 2 cm in diameter), firm, and nontender

    • Persist for days or weeks and are recurrent

    • Attached to fascia or tendon sheaths over bony prominences

    • Indistinguishable from rheumatoid nodules

    • Uncommon except in children

  • Sydenham chorea

    • Involuntary choreoathetoid movements primarily of the face, tongue, and upper extremities

    • May be the sole manifestation of rheumatic fever; only half of cases have other overt signs

    • Girls are more frequently affected; occurrence in adults is rare

    • Least common (3% of cases) but most diagnostic manifestation of acute rheumatic fever

  • Polyarthritis


Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.