Signs and Symptoms of Rheumatic Fever
Rheumatic fever presents with a constellation of clinical manifestations including carditis, arthritis, chorea, skin findings, and constitutional symptoms, with diagnosis based on the 2015 revised Jones criteria requiring evidence of preceding group A streptococcal infection plus specific major and minor criteria. 1, 2
Major Manifestations
Carditis (50-70% of cases)
- Clinical carditis: Auscultatory findings of mitral or aortic valve regurgitation
- Subclinical carditis: Detected by echocardiography/Doppler without clinical findings
Arthritis (35-66% of cases)
- Typically migratory polyarthritis affecting large joints
- Most commonly involves knees, wrists, and ankles
- Can also affect elbows, shoulders, hips, and small joints
- Symmetric pattern with joint pain associated with fever spikes
- In high-risk populations, monoarthritis or polyarthralgia may be considered 1
Sydenham's Chorea (10-30% of cases)
- Purposeless, involuntary, nonstereotypical movements
- Associated with muscle weakness and emotional lability
- More common in females
- May be unilateral
- Often appears later than other manifestations
- May occur without evidence of preceding streptococcal infection due to long latent period 1
Skin Manifestations
Erythema marginatum (<6% of cases)
- Evanescent, pink rash with pale centers and rounded/serpiginous margins
- Located on trunk and proximal extremities, not facial
- Heat can induce appearance; blanches with pressure
- May be harder to detect in dark-skinned individuals 1
Subcutaneous nodules (0-10% of cases)
- Firm, painless protuberances on extensor surfaces
- Found at knees, elbows, wrists, occiput, and along vertebrae
- Often observed in patients who also have carditis
- Rarely occur as sole major manifestation 1
Minor Manifestations
Fever
- In low-risk populations: ≥38.5°C (101.3°F)
- In high-risk populations: ≥38°C (100.4°F)
- Often transient, lasting under 4 hours
- Typically quotidian or double quotidian pattern
- Highest temperatures in late afternoon/early evening 1, 2
Laboratory Findings
- Elevated acute phase reactants:
- Prolonged PR interval on ECG 2
Other Minor Symptoms
- Polyarthralgia (if not counted as major criterion)
- Sore throat (often preceding other symptoms)
- Abdominal pain
- Rapid sleeping pulse rate
- Tachycardia out of proportion to fever
- Malaise
- Anemia
- Leukocytosis
- Epistaxis
- Precordial pain 1
Diagnostic Requirements
Initial Attack
- Low-risk populations: 2 major OR 1 major + 2 minor criteria PLUS evidence of preceding GAS infection
- Moderate/high-risk populations: Lower threshold for diagnosis with modified criteria 2
Recurrent Attack
- With history of ARF/RHD and documented GAS infection: 2 major OR 1 major + 2 minor OR 3 minor manifestations 1
Evidence of Preceding Streptococcal Infection
- Positive throat culture for group A streptococcus
- Positive rapid streptococcal antigen test
- Elevated or rising streptococcal antibody titers (ASO, anti-DNase B) 1, 2
Clinical Pearls and Pitfalls
- Carditis is the most serious manifestation due to potential long-term valve damage
- Arthritis is typically responsive to anti-inflammatory medications
- Normal ESR and CRP should prompt reconsideration of diagnosis (except in isolated chorea)
- Subclinical carditis detected by echocardiography is now considered a major criterion
- Chorea may appear weeks to months after the streptococcal infection when other manifestations have resolved 1
- Skin manifestations (erythema marginatum and subcutaneous nodules) rarely occur as stand-alone major criteria 2
Early recognition of these signs and symptoms is crucial for prompt diagnosis and treatment to prevent long-term cardiac complications.