Acute rheumatic fever
Acute rheumatic fever is a nonsuppurative, immune-mediated multisystem illness that follows group A streptococcal (GAS) pharyngitis, driven by antibodies against the streptococcal M protein that cross-react with cardiac, joint, skin, and CNS tissue. It is diagnosed using the JONES major criteria with evidence of antecedent GAS infection, and treated with antibiotics to eradicate the pharyngitis plus salicylates or corticosteroids for inflammation, followed by secondary penicillin prophylaxis to prevent rheumatic heart disease.
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What this article covers
- Overview
A delayed immune-mediated illness following streptococcal pharyngitis, not a direct infection: acute rheumatic fever is a nonsuppurative consequence of pharyngeal group A beta-hemolytic streptococcal…
- Epidemiology
Follows GAS pharyngitis specifically: a delayed complication of untreated or inadequately treated group A streptococcal pharyngitis; only 5% to 15% of pharyngitis cases overall are caused by GAS.
- Risk factors
Untreated or inadequately treated group A streptococcal pharyngitis: the essential antecedent event; antibiotic treatment is the single most effective way to prevent the first episode.
- Pathophysiology
Molecular mimicry drives a type II hypersensitivity reaction: antibodies against the streptococcal M protein cross-react with self-antigens, most notably cardiac myosin, because the M protein shares…
- Clinical features
This disease establishes its own diagnostic criteria through its clinical features (the JONES major criteria); combining these findings with evidence of antecedent GAS infection makes the diagnosis…
- Differential diagnosis
Infective endocarditis: bacterial infective endocarditis causes fever and a new/changing murmur overlapping with rheumatic carditis; positive blood cultures and Duke criteria vegetations confirm it…
- Diagnosis
Acute rheumatic fever is diagnosed using the Jones criteria framework: two major manifestations, or one major plus two minor manifestations, plus evidence of a preceding group A streptococcal infection.
- Management
First, treat the antecedent group A streptococcal infection with antibiotics, even if the throat culture is now negative: oral penicillin V 500 mg PO every 8-12 hours for 10 days, or a single dose of…
- Complications
Rheumatic heart disease: the dominant long-term complication, principally mitral regurgitation evolving over years into mitral stenosis, with the aortic valve affected next most and the tricuspid…
- Prognosis
Acute manifestations other than carditis typically resolve completely: arthritis, erythema marginatum, subcutaneous nodules, and chorea generally resolve without permanent sequelae.
- Prevention
Primary prevention: treat group A streptococcal pharyngitis promptly with antibiotics (penicillin or amoxicillin) in any patient with 3 or more Centor criteria (fever, tonsillar exudates, tender…
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Related articles
- Streptococcal pharyngitis
Streptococcal pharyngitis is an acute infection of the tonsils and pharynx caused by group A Streptococcus pyogenes, presenting with rapid-onset sore throat, fever, tonsillar exudates, and tender anterior cervical lymphadenopathy without cough; diagnosis relies on the Centor criteria to guide rapid antigen testing, and treatment with penicillin or amoxicillin prevents suppurative complications and acute rheumatic fever.
- Bacterial infective endocarditis
Bacterial infection of the endocardial surface, usually a heart valve, producing vegetations that destroy the valve and embolize; diagnosis rests on the modified Duke criteria (blood cultures plus echocardiography) and treatment is a prolonged organism-directed intravenous antibiotic course, with valve surgery for heart failure, uncontrolled infection, or embolic recurrence.