Rheumatic Fever

Definition:
Rheumatic fever is a multisystem inflammatory disease that occurs as a sequel to
pharyngeal infection with the bacterium, Group A Streptococcus pyogenes. Major
features are arthritis and carditis.

Clinical Features:
These are varied. They include fever, pain (with or without swelling) in one or more
joints, endocarditis, pericarditis, myocarditis, pleurisy, subcutaneous nodules, a
characteristic skin lesion (erythema marginatum) and an even more characteristic
disturbance of central nervous system function, Sydenham’s chorea. Carditis is
more frequent in the youngest age groups, and the majority of rheumatic fever
attacks occurring in adults are manifested primarily by arthritis.

1. Arthritis usually affects larger joints, particularly wrists, elbows, knees and ankles.
   Hips are less often affected, small joints of hands and feet rarely, and spine almost
   never. Characteristically, fleeting arthritis occurs but more commonly, only arthralgia alone occurs. Untreated, the joint pains settle within 1-4 weeks. The
   arthritis of rheumatic fever does not lead to permanent damage to the joints
   affected.
2. Carditis is the most important clinical manifestation of rheumatic fever, being the
   one with permanent effects. Endocarditis is detected clinically by new or changing murmurs, and pericarditis by a friction rub. Heart failure is the most serious presentation,
   occurring in younger patients.
3. Chorea is now rare. The latent period is 1 to 6 months. It features jerky, purposeless
   movements, exaggerated by tension but disappearing in sleep. It occurs
   predominantly in females.
4. Other less common manifestations include erythema marginatum, an evanescent
   macular eruption and subcutaneous nodules, which may
   appear as painless lumps in people with long-standing carditis.

Recurrence:

An attack of rheumatic fever greatly increases the chances that a subsequent
streptococcal throat infection will be followed by another attack of rheumatic
fever. The risk of recurrence is greater in children, in patients with pre-existing
rheumatic heart disease, and in those experiencing symptomatic throat infection.
The risk declines with advancing age, but nevertheless rheumatic patients remain at
increased risk well into adult life.

Diagnosis:

Revised Jones' criteria - evidence of preceding strep infection (positive throat culture or rapid streptococcal antigen test, increased or rising anti-strep Ab, recent scarlet fever) plus 2 major or 1 major and 2 minor criteria.

Major criteria

1. Polyarthritis
2. Carditis
3. Chorea
4. Subcutaneous nodules
5. Erythema marginata

Minor criteria

1. History of acute rheumatic fever
2. Rheumatic heart disease
3. Fever
4. Arthralgias
5. Increased WBC, increased ESR, increased C-reactive protein
6. Prolonged PR interval on ECG

Complications:

The major complication of rheumatic fever is valvular disease of the heart giving rise to pansystolic blowing mitral murmur, less commonly diastolic aortic murmur at left sternal border.

Treatment:

• Treat streptococcal infection with antibiotics : penicillin G 600,000-1.2 million units as 1 injection or PO for 10 days
• Erythromycin if allergic to penicillin
• Anti-inflammatory treatment - aspirin is treatment of choice for ARF
• Sedating drugs for severe chorea - phenobarbital, chlorpromazine, haloperidol