Study note

Jones criteria and clinical manifestations of rheumatic fever

THE CONTEXT
The supplied Jones criteria distinguish major and minor manifestations and require attention to evidence of preceding streptococcal infection.

Major and minor manifestations

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Major manifestations are carditis, polyarthritis, chorea, erythema marginatum and subcutaneous nodules.
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Minor manifestations are fever, arthralgia, raised erythrocyte sedimentation rate or C-reactive protein, previous rheumatic fever, leucocytosis and first-degree atrioventricular block.

Evidence of preceding infection

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Supporting evidence includes recent scarlet fever, a raised antistreptolysin O or other streptococcal antibody titre, or a positive throat culture.
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Evidence of recent streptococcal infection is particularly important when there is only one major manifestation.

Carditis

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Rheumatic fever causes pancarditis involving the endocardium, myocardium and pericardium to varying degrees; this is more commonly seen in younger patients.
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Breathlessness may result from myocardial dysfunction, valve dysfunction or pericardial effusion. Palpitations and chest pain may also occur; pain may be due to pericarditis, pleurisy or pancarditis.
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Other findings include tachycardia, cardiac enlargement and new or changed murmurs. Mitral regurgitation and stenosis can produce systolic and diastolic mitral murmurs, respectively.
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Aortic regurgitation occurs in 50% of cases, while tricuspid and pulmonary valve involvement is rare.
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Electrocardiograms may show nonspecific ST and T-wave changes. Conduction defects, including symptomatic atrioventricular block, can occur.

Arthritis

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Arthritis is the commonest major manifestation and occurs early, when streptococcal antibody titres are high.
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It is an acute, painful, asymmetric and migratory inflammation of large joints, typically affecting the knees, ankles, elbows and wrists.

Skin manifestations

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Erythema marginatum occurs in fewer than 5% of patients. Red macules fade centrally but remain red at their edges, mainly affecting the trunk and proximal extremities while sparing the face.
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Subcutaneous nodules occur in 5%–7% of patients. They are small, measuring 0.5–2.0 cm, firm and painless.
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Nodules are best felt over extensor surfaces of bone or tendons and typically appear after other clinical features.

Sydenham chorea

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Sydenham chorea, also called St Vitus dance, is a late neurological manifestation appearing at least 3 months after acute rheumatic fever, when other signs may have disappeared.
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