What is Rheumatic Fever & Rheumatic Heart Disease

Rheumatic fever is an inflammatory disease which may develop after an infection with streptococcus bacteria (such as strep throat or scarlet fever) and can involve the heart (especially the valves), joints, skin, and brain.

Statistics on Rheumatic Fever & Rheumatic Heart Disease

Rare in developed countries such as the UK, the US and Australia. Incidence has declined from 10% of children in the 1920’s to 0.01% of children today. This drop in incidence reflects advances in infection control and treatment of streptococcal infections with antimicrobials.

The disease is still common in developing nations such as the Middle and Far East and South America.

Risk Factors for Rheumatic Fever & Rheumatic Heart Disease

Rheumatic fever is preceded by a throat infection with group A streptococcus organsisms.

Progression of Rheumatic Fever & Rheumatic Heart Disease

Following a group A streptococcus pharyngeal infection, usually in childhood (5-15yo) is followed by a systemic syndrome thought to be caused by an autoimmune response triggered by the infection.

The systemic syndrome includes:
1) Polyarthritis (multiple join pain and inflammation)
2) Carditis (heart inflammation)
3) Skin manifestations
4) Nervous system manifestations
5) Fever, joints pains etc.

About 50% of patients who develop carditis during the initial phase of the illness will go on to develop long term rheumatic heart disease and associated valvular heart disease.

How is Rheumatic Fever & Rheumatic Heart Disease Diagnosed?

1) Blood tests: ESR and CRP (non-specific markers of inflammation), leukocytosis
2) Chest x-ray: may show evidence of carditis, pericarditis, pericardial effusion.
3) ECG: may show evidence of carditis (prolonged P-R interval) or of pericarditis (saddle shaped ST segment elevations).

Prognosis of Rheumatic Fever & Rheumatic Heart Disease

Some parts of the acute clinical syndrome may recur after the intial episode. However the prognosis for the joint disease, skin disease and nervous system disease associated with rheumatic fever is excellent with no known long term sequelae.

Rheumatic heart disease has traditionally been hailed the major causative factor in valvular heart disease and carries the associated morbidity and mortality.

How is Rheumatic Fever & Rheumatic Heart Disease Treated?

  • Residual strep infection should be treated with a single intramuscular dose of 916mg of benzylpenicillin.
  • Rest is important.
  • The acute syndrome is treated with high dose aspirin therapy to the limit of tolerance as judged by the development of tinnitus.
  • If carditis is present, steroid therapy may play a role in reducing progression to chronic rheumatic heart disease.
  • Subsequent strep infections should be treated promptly.

Rheumatic Fever & Rheumatic Heart Disease References

[1] Hurst’s The Heart 8th Edition, McGRAW-HILL 1994.
[2] Kumar and Clark Clinical Medicine 4th Edition, W.B SAUNDERS 1998.
[3] MEDLINE Plus.