Symptoms of Rheumatic Fever
Rheumatic fever presents with a characteristic constellation of symptoms including migratory polyarthritis (joint pain moving from joint to joint), carditis (heart inflammation), chorea (involuntary movements), subcutaneous nodules, and erythema marginatum (a distinctive rash), occurring in varying combinations 2–4 weeks after untreated group A streptococcal pharyngitis. 1, 2
Major Clinical Manifestations
The classic symptoms of rheumatic fever are defined by the Jones Criteria and include:
Migratory polyarthritis – Joint pain and swelling that moves from one large joint to another (knees, ankles, elbows, wrists), representing the most common major manifestation. 1, 2
Carditis – Heart inflammation that may involve the pericardium, myocardium, or endocardium; this is the most serious manifestation and can range from mild to fatal in severe cases. 1, 2
Sydenham chorea – Involuntary, purposeless movements of the extremities and face, often accompanied by emotional lability; this neurological manifestation may appear months after the initial streptococcal infection. 1
Subcutaneous nodules – Firm, painless nodules over bony prominences or extensor tendons, though these are relatively uncommon. 1, 2
Erythema marginatum – A distinctive pink or faint red rash with clear centers and rounded or serpiginous margins, typically on the trunk and proximal extremities but not the face. 1, 2
Minor Clinical Manifestations
Additional symptoms that support the diagnosis include:
Fever – Typically present during the acute phase of illness. 1, 2
Arthralgia – Joint pain without objective swelling (when polyarthritis is not present as a major criterion). 2
Prolonged PR interval on electrocardiogram, indicating cardiac conduction abnormalities. 2
Elevated acute-phase reactants (ESR, CRP) reflecting systemic inflammation. 2
Temporal Relationship and Age Distribution
Rheumatic fever occurs as a delayed sequela 2–4 weeks after group A streptococcal pharyngitis, following a characteristic latency period. 1, 3
The disease most commonly affects children aged 5–15 years, with peak incidence in school-age children. 4, 2
Rheumatic fever is exceedingly rare in children younger than 3 years in the United States, though exceptional cases have been reported in toddlers following severe streptococcal infections. 4, 5
Preceding Streptococcal Infection
At least one-third of rheumatic fever cases result from inapparent (asymptomatic) streptococcal infections, meaning the patient may not recall having a sore throat. 4
When symptomatic, the preceding pharyngitis typically presented with sudden-onset sore throat, fever (101–104°F), pain on swallowing, tonsillopharyngeal erythema with or without exudates, and tender anterior cervical lymphadenopathy. 4, 6
Critical Clinical Pitfalls
Rheumatic fever symptoms appear weeks after the throat infection has resolved, so patients and families may not connect the two events. 1, 3
Chorea may appear months after the initial infection and can occur as an isolated manifestation without other Jones criteria, making diagnosis particularly challenging. 1
In very young children (toddlers), the presentation may be atypical and the applicability of Jones criteria is debatable, potentially leading to diagnostic uncertainty. 5
Carditis may be subclinical initially but progress to chronic rheumatic heart disease, the most important long-term complication and leading cause of cardiovascular mortality in endemic regions. 1, 7
Natural History
In its classic milder form, rheumatic fever is largely self-limited and resolves without sequelae over weeks to months. 1
However, chronic and progressive valvular damage (rheumatic heart disease) represents the most serious public health consequence, developing in a significant proportion of patients with carditis. 1, 7
Anti-inflammatory treatment provides dramatic symptomatic improvement but does not prevent the subsequent development of rheumatic heart disease. 1