What are the major and minor Jones criteria for acute rheumatic fever?

Jones Criteria for Acute Rheumatic Fever

The 2015 revised Jones Criteria from the American Heart Association require documented evidence of recent group A streptococcal infection PLUS either 2 major manifestations OR 1 major and 2 minor manifestations, with different thresholds based on population risk. 1

Diagnostic Requirements

All patients must have evidence of preceding group A streptococcal (GAS) infection documented by: 1

• Positive throat culture or rapid antigen detection test
• Elevated or rising anti-streptolysin O (ASO) titer
• Elevated anti-DNase B titer (combined ASO and anti-DNase B testing detects up to 98% of proven streptococcal cases)

Then apply the following combinations: 1

• Initial ARF: 2 major manifestations OR 1 major + 2 minor manifestations
• Recurrent ARF: 2 major OR 1 major + 2 minor OR 3 minor manifestations

Population Risk Stratification

Low-risk populations are defined as ARF incidence ≤2 per 100,000 school-aged children per year OR rheumatic heart disease prevalence ≤1 per 1,000 population per year. 1

Moderate-to-high-risk populations exceed these thresholds and require more sensitive (but less specific) diagnostic criteria. 1

Major Criteria

For Low-Risk Populations:

• Carditis (clinical and/or subclinical detected by echocardiography) 1
• Polyarthritis only (monoarthritis does NOT qualify) 1
• Chorea (Sydenham's chorea) 1
• Erythema marginatum (evanescent pink rash with pale centers and rounded/serpiginous margins on trunk and proximal extremities, NOT facial, blanches with pressure, heat can induce appearance) 1, 2
• Subcutaneous nodules (firm, painless protuberances over bony prominences including knees, elbows, wrists, occiput, and spinous processes) 1, 2

For Moderate-to-High-Risk Populations (includes all above PLUS):

• Monoarthritis (in addition to polyarthritis) 1
• Polyarthralgia (after exclusion of other causes) 1

Critical pitfall: Joint manifestations can only be counted as EITHER a major OR minor criterion, never both in the same patient. 1 Erythema marginatum and subcutaneous nodules rarely appear as isolated major criteria and are almost always accompanied by carditis. 1, 2

Minor Criteria

For Low-Risk Populations:

• Polyarthralgia 1
• Fever ≥38.5°C 1
• Elevated inflammatory markers: ESR ≥60 mm/h OR CRP ≥3.0 mg/dL (use peak ESR values as they evolve during ARF course) 1
• Prolonged PR interval (after accounting for age variability, unless carditis is already a major criterion) 1

For Moderate-to-High-Risk Populations:

• Monoarthralgia (lower threshold than low-risk) 1
• Fever ≥38°C (lower threshold) 1
• Elevated inflammatory markers: ESR ≥30 mm/h OR CRP ≥3.0 mg/dL (lower threshold) 1
• Prolonged PR interval (unless carditis is already a major criterion) 1

Echocardiographic Criteria for Subclinical Carditis

This represents a major change from 1992 criteria—subclinical carditis detected by Doppler echocardiography now counts as a major manifestation. 1

Pathological Mitral Regurgitation:

• Jet visible in ≥2 planes 1
• Length >1 cm 1
• Holosystolic 1
• Peak velocity >2.5 m/s 1

Pathological Aortic Regurgitation:

• Jet visible in ≥2 planes 1
• Holodiastolic 1
• Peak velocity >2.5 m/s 1

Critical pitfall: Measure blood pressure at the time of echocardiography because systemic pressure influences jet characteristics and can lead to misdiagnosis. 1 Signals with peak velocity <3.0 m/s are more likely physiological regurgitation. 1 You must differentiate rheumatic valvulitis from physiological regurgitation (common in normal children), mitral valve prolapse, congenital valve anomalies (bicuspid aortic valve, cleft mitral valve), and infective endocarditis. 1

Special Diagnostic Scenarios

Recurrent Rheumatic Fever (Prior ARF or RHD):

In patients with documented prior ARF or established RHD and confirmed recent GAS infection, the threshold is lowered to: 1

• 2 major manifestations, OR
• 1 major + 2 minor manifestations, OR
• 3 minor manifestations

"Possible" Rheumatic Fever (High Clinical Suspicion but Incomplete Criteria):

When clinical presentation doesn't fulfill Jones Criteria but suspicion remains high: 3

• Offer 12 months of secondary prophylaxis with benzathine penicillin
• Arrange repeat detailed history, physical examination, and echocardiography at 12 months
• If recurrent joint symptoms occur in a patient adherent to prophylaxis but WITHOUT serologic evidence of GAS infection and WITHOUT echocardiographic valvulitis, the symptoms are likely unrelated to ARF and discontinuation of prophylaxis may be appropriate

Key Differential Diagnoses to Exclude:

Always consider and exclude: 1, 4

• Post-streptococcal reactive arthritis (PSRA)—prolonged arthritis that fails to respond promptly to salicylates, develops 3-14 days after pharyngitis
• Lyme disease
• Serum sickness and drug reactions
• Infective endocarditis
• Congenital valve abnormalities
• Viral myocarditis
• Systemic lupus erythematosus