Rheumatic Fever

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

Demographics

• Rheumatic fever

  • Rare before age 4 or after age 40 years

  • Responsible for 250,000 deaths in young people worldwide each year

  • Over 15 million have evidence for rheumatic heart disease

• Rheumatic heart disease

  • While there has been progress against this disease, it remains a major cardiovascular problem in the poorest regions of the world

  • In 2015, there were 33.4 million cases globally

  • Responsible for 320,000 deaths in 2015

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 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

  • Involves the large joints sequentially

  • The arthritis lasts 1–5 weeks and subsides without residual deformity

  • In adults, only a single joint may be affected

Minor Criteria

• Fever

• Polyarthralgias

• Reversible prolongation of the PR interval

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

• Positive throat culture or rapid streptococcal antigen test and elevated or rising streptococcal antibody titer

• Rapid ESR is nonspecific evidence of inflammatory disease

• High or increasing titers of antistreptococcal antibodies (antistreptolysin O and anti-DNase B)

General Measures

• Bed rest until the temperature, resting pulse rate, ESR and ECG have returned to normal

Medications

• Salicylates

  • Adults may require large doses of aspirin, 0.6–0.9 g every 4 hours; children, lower doses

  • Markedly reduce fever, relieve joint pain and swelling, but have no effect on the natural course of the disease

• Penicillin

  • Benzathine penicillin, 1.2 million units intramuscularly once, or procaine penicillin, 600,000 units intramuscularly daily for 10 days) to eradicate streptococcal infection

  • Erythromycin may be substituted (40 mg/kg/day)

• Corticosteroids

  • Prednisone, 40–60 mg orally daily, with tapering over 2 weeks

    • Usually causes rapid improvement of the joint symptoms

    • Indicated when response to salicylates has been inadequate

Complications

• Rheumatic valvular disease affects

  • The mitral valve most commonly

  • The aortic valve second most commonly

  • The tricuspid valve third most commonly

• Heart failure

• Arrhythmias, pericarditis with effusion, and rheumatic pneumonitis

Prevention

• Initial episode of rheumatic fever can usually be prevented by early treatment of streptococcal pharyngitis with penicillin

• Prevention of recurrent episodes is critical

  • Preferred: Benzathine penicillin G, 1.2 million units intramuscularly every 4 weeks

  • Less reliable: Oral penicillin (200,000–250,000 units twice daily)

  • Penicillin allergy: Sulfadiazine (or sulfisoxazole), 1 g daily; or erythromycin, 250 mg orally twice daily

Prognosis

• Initial episodes may last months in children and weeks in adults

• The immediate mortality rate is 1–2%

• Persistent rheumatic carditis with cardiomegaly, heart failure, and pericarditis implies a poor prognosis