Acute rheumatic fever

Jonathan R. Carapetis

in Oxford Textbook of Medicine

Fifth edition

Published on behalf of Oxford University Press

Published in print May 2010 | ISBN: 9780199204854
Published online May 2010 | e-ISBN: 9780199570973 | DOI:

Series: Oxford Textbooks

Acute rheumatic fever

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Acute rheumatic fever is an immunologically mediated multisystem disease induced by recent infection with group A streptococcus. About 5% of people have the potential to develop acute rheumatic fever after infection by a strain of streptococcus with propensity to cause the condition. Most cases (97%) occur in developing countries, particularly in sub-Saharan Africa, Pacific nations, Australasia, and the Indian subcontinent. Children aged 5 to 15 years are most commonly affected, and rheumatic heart disease remains the most common acquired heart disease of childhood in the world.

Presentation—after a latent period (1–5 weeks in most cases, but up to 6 months for presentation with chorea) the disease presents with one or more of the following major criteria: (1) carditis—most typically manifest as an apical pansystolic murmur of mitral regurgitation; (2) polyarthritis—severe, large-joint, and migratory; (3) chorea; (4) subcutaneous nodules; (5) erythema marginatum. Other minor criteria that can support the diagnosis include fever, polyarthralgia, elevated acute phase reactants or neutrophil count, prolongation of the PR interval on the ECG.

Diagnosis—in addition to the criteria described above, evidence of preceding group A streptococcal infection is required: (1) positive throat culture, or (2) elevated or rising anti-Streptolysin O or other streptococcal antibody, or (3) rapid antigen test for group A streptococcus, or (4) recent scarlet fever (contentious).

Prognosis and management—untreated acute rheumatic fever lasts for about 3 months. All patients with acute disease should be given penicillin to eradicate the group A streptococcus that precipitated the attack. Children with arthritis or severe arthralgia should be treated with nonsteroidal anti-inflammatory medication (usually salicylates). For severe carditis, many clinicians use oral prednisone or prednisolone at a dose of 40 to 60 mg/day (1–2 mg/kg per day in children), tapering after 2 or 3 weeks, but benefit is not proven. Important prognostic factors are the severity of the acute carditis and the number of recurrences: 30 to 50% of patients with a first episode of acute rheumatic fever will develop chronic rheumatic heart disease, but more than 70% of those with severe carditis at the first episode, or with recurrent episodes.

Secondary prophylaxis—every patient with acute rheumatic fever should immediately commence intramuscular benzathine penicillin G every 3 or 4 weeks, or twice daily oral penicillin V. In patients without carditis, this should continue for 5 years or until age 18, whichever comes later; with mild or healed carditis, for 10 years or until age 25, whichever is longer; those with more severe valvular disease or after valve surgery should have secondary prophylaxis for life.

Primary prophylaxis—a full course of penicillin treatment commencing within 9 days of the onset of symptomatic group A streptococcal pharyngitis will prevent the subsequent development of acute rheumatic fever in most cases, but this is not widely practised in most developing countries and would not prevent those cases of acute rheumatic fever which do not follow a sore throat.

Chapter.  7946 words.  Illustrated.

Subjects: Cardiovascular Medicine

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