What are the Jones criteria for diagnosing rheumatic fever and how is the diagnosis confirmed?

Diagnosing Rheumatic Fever Using the Revised Jones Criteria

Acute rheumatic fever is diagnosed using the 2015 revised Jones Criteria, which requires documented evidence of preceding group A streptococcal (GAS) infection PLUS either 2 major manifestations OR 1 major and 2 minor manifestations, with different thresholds depending on whether the patient is from a low-risk versus moderate-to-high-risk population. 1

Evidence of Preceding GAS Infection (Required for All Cases)

Before applying the Jones Criteria, you must document recent streptococcal infection through: 2

• 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, making dual testing the preferred approach. 2

Population Risk Stratification

Low-risk populations are defined as those with ARF incidence ≤2 per 100,000 school-aged children 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 by Population Risk

For Low-Risk Populations: 1

• Carditis (clinical and/or subclinical detected by echocardiography)
• Polyarthritis only (monoarthritis does NOT qualify)
• Chorea
• Erythema marginatum (evanescent pink rash with pale centers on trunk/proximal extremities, not face, blanches with pressure) 3
• Subcutaneous nodules (firm, painless nodules over bony prominences: knees, elbows, wrists, occiput, spinous processes) 3

For Moderate-to-High-Risk Populations: 1

• Carditis (clinical and/or subclinical)
• Monoarthritis OR polyarthritis
• Polyarthralgia (after excluding other causes)
• Chorea
• Erythema marginatum
• Subcutaneous nodules

Minor Criteria by Population Risk

For Low-Risk Populations: 1

• Polyarthralgia
• Fever ≥38.5°C
• ESR ≥60 mm/h and/or CRP ≥3.0 mg/dL
• Prolonged PR interval (unless carditis is already a major criterion)

For Moderate-to-High-Risk Populations: 1

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

Role of Echocardiography

Subclinical carditis detected by Doppler echocardiography now qualifies as a major manifestation, representing a critical change from the 1992 criteria. 2 Echocardiography should be performed in all suspected cases. 2

Echocardiographic Criteria for Pathological Regurgitation: 4, 2

For Mitral Regurgitation:

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

For Aortic Regurgitation:

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

Critical pitfall: Measure blood pressure at the time of echocardiography, as systemic pressure affects jet characteristics and can lead to misdiagnosis. 1 Distinguish rheumatic valvulitis from physiological regurgitation (signals <3.0 m/s peak velocity are more likely physiological), mitral valve prolapse, congenital valve abnormalities, and infective endocarditis. 1

Special Diagnostic Scenarios

Recurrent Rheumatic Fever

In patients with documented prior ARF or rheumatic heart disease AND confirmed GAS infection, the diagnostic threshold is lower: 1, 2

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

When only minor manifestations are present, exclude other causes before diagnosing recurrence. 1

"Possible" Rheumatic Fever

When clinical presentation doesn't fulfill Jones Criteria but suspicion remains high (particularly in high-incidence settings with limited laboratory access or unclear documentation): 1

• Offer 12 months of secondary prophylaxis
• Reevaluate with detailed history, physical examination, and repeat echocardiogram

If recurrent joint symptoms occur in a patient adherent to prophylaxis but lacking serological evidence of GAS infection and echocardiographic valvulitis, the symptoms are likely not ARF-related and discontinuation of prophylaxis may be appropriate. 1

Key Diagnostic Pitfalls

Joint manifestations can only count in either the major OR minor category, never both in the same patient. 1

Erythema marginatum and subcutaneous nodules rarely occur as stand-alone major criteria and are almost always accompanied by other manifestations, particularly carditis. 1, 3

Distinguish from mimics: Post-streptococcal reactive arthritis, Lyme disease, serum sickness, drug reactions, infective endocarditis, and congenital valve abnormalities can all resemble ARF. 1, 5