What are the signs and symptoms of rheumatic fever?

Signs and Symptoms of Rheumatic Fever

Rheumatic fever presents with a classic triad of high-spiking fever, migratory polyarthritis, and carditis, along with less common but highly specific manifestations including chorea, erythema marginatum, and subcutaneous nodules. 1

Major Manifestations (Jones Criteria)

Carditis (50-70% of cases)

• Most common major manifestation during first episodes of acute rheumatic fever 1
• Diagnosed clinically by auscultation of typical cardiac findings, though echocardiography with Doppler has become increasingly important in the modern era 1
• Can result in rheumatic heart disease with long-term valvular damage 2
• More frequently associated with subcutaneous nodules when present 1, 3

Arthritis (35-66% of cases)

• Migratory polyarthritis is the classic presentation, affecting multiple joints sequentially 1, 4
• Most commonly involves knees, wrists, and ankles, though elbows, shoulders, and small joints of hands and feet can be affected 1
• Pattern is typically symmetric polyarthritis with joint pain associated with fever spikes 1
• In high-risk populations (such as indigenous Australians), aseptic monoarthritis or polyarthralgia alone may be sufficient for diagnosis 1

Chorea (Sydenham Chorea) (10-30% of cases)

• Purposeless, involuntary, nonstereotypical movements of trunk or extremities 1
• Associated with muscle weakness and emotional lability 1
• Shows female predominance 1
• Can be predominantly unilateral, requiring careful neurological examination 1
• Evidence of recent streptococcal infection may be difficult to document due to long latent period between infection and chorea onset 1

Erythema Marginatum (<6% of cases)

• Unique, evanescent pink rash with pale centers and rounded or serpiginous margins 1, 3
• Present on trunk and proximal extremities, not facial 1, 3
• Heat can induce its appearance and it blanches with pressure 1, 3
• May be harder to detect in dark-skinned individuals 1
• Almost never occurs as sole major manifestation 1, 3

Subcutaneous Nodules (0-10% of cases)

• Firm, painless protuberances found on extensor surfaces 1, 3
• Located at specific joints: knees, elbows, wrists, occiput, and along spinous processes of thoracic and lumbar vertebrae 1, 3
• More often observed in patients who also have carditis 1, 3
• Almost never occur as sole major manifestation 1, 3
• Show no racial or population variability 1, 3

Minor Manifestations (Supporting Criteria)

Fever

• Temperature ≥38.5°C (101.3°F) in low-risk populations 5
• Temperature ≥38°C (100.4°F) in moderate- and high-risk populations 5
• Develops 2-3 weeks after group A streptococcal pharyngitis (typically 14-21 days post-infection) 5
• In high-risk populations, lower-grade fevers should not prompt dismissal of diagnosis 5
• Widespread availability of antipyretic agents requires detailed history to contextualize fever presentation 1

Elevated Inflammatory Markers

• Erythrocyte sedimentation rate (ESR) ≥60 mm/hr in low-risk populations or ≥30 mm/hr in moderate-/high-risk populations 1, 5
• C-reactive protein (CRP) ≥3.0 mg/dL (above upper limit of normal), commonly >7.0 mg/dL depending on laboratory method 1, 5
• Normal ESR and CRP prompt serious reconsideration of acute rheumatic fever diagnosis, except in patients with isolated chorea 1

Prolonged PR Interval on ECG

• Standard minor criterion without population variability 1

History of Previous Rheumatic Fever or Rheumatic Heart Disease

• Increases risk for recurrent episodes 1

Additional Clinical Features (Not Part of Jones Criteria)

• Abdominal pain 1
• Rapid sleeping pulse rate and tachycardia out of proportion to fever 1
• Malaise, anemia, leukocytosis 1
• Epistaxis and precordial pain 1
• Myalgias (frequently noted but not diagnostic) 1

Critical Diagnostic Context

Diagnosis requires documented preceding group A streptococcal infection plus either two major manifestations or one major and two minor manifestations from the Jones criteria 6. Without documentation of antecedent streptococcal infection, diagnosis is much less likely except in rare scenarios 6. The diagnosis remains entirely clinical without any laboratory gold standard 2, 6.