Rheumatic Fever

Key Features

Essentials of Diagnosis

• 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

General Considerations

• 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

Clinical Findings

• 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

Diagnosis

Jones criteria (modified)

• 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

Treatment

• 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 ...