Jones Criteria for Diagnosing Acute Rheumatic Fever
The diagnosis of acute rheumatic fever requires documented evidence of preceding Group A Streptococcal infection PLUS either 2 major manifestations OR 1 major and 2 minor manifestations, with different diagnostic thresholds applied based on whether the population is at low-risk versus moderate-to-high-risk for rheumatic fever. 1
Evidence of Preceding Streptococcal Infection (Required)
You must document recent Group A Streptococcal infection through one of the following 1:
- Positive throat culture or rapid antigen detection test 1
- Elevated or rising anti-streptolysin O (ASO) titer 1
- Elevated anti-DNase B titer 1
Combined ASO and anti-DNase B testing detects up to 98% of proven streptococcal cases, making dual testing the preferred approach when initial testing is negative but clinical suspicion remains high 1.
Major Manifestations
For Low-Risk Populations
- Carditis (clinical or subclinical detected by echocardiography) 2, 1
- Polyarthritis (migratory inflammation of multiple joints) 1
- Chorea (Sydenham's chorea) 3
- Erythema marginatum (characteristic rash) 1
- Subcutaneous nodules 1
For Moderate-to-High-Risk Populations
The major manifestations are expanded to include 1:
- Monoarthritis (in addition to polyarthritis) 1
- Polyarthralgia (after excluding other causes) 1
- All other major criteria listed above 1
Minor Manifestations
For Low-Risk Populations
- Fever ≥38.5°C 2
- Polyarthralgia 1
- Elevated acute phase reactants (ESR or CRP) 2
- Prolonged PR interval on ECG 2
For Moderate-to-High-Risk Populations
- Fever ≥38.0°C (lower threshold) 2
- Monoarthralgia (in addition to polyarthralgia) 1
- Elevated acute phase reactants 2
- Prolonged PR interval on ECG 2
Critical Role of Echocardiography
Subclinical carditis detected by Doppler echocardiography is now accepted as a major manifestation, representing the most significant change from the 1992 criteria. 1 This modification is crucial because many patients have valve involvement without audible murmurs 2.
Echocardiographic Criteria for Pathological Regurgitation
For mitral regurgitation 4:
- Jet visible in 2 planes
- Length >1 cm
- Holosystolic
- Peak velocity >2.5 m/s
For aortic regurgitation 4:
- Jet visible in 2 planes
- Holodiastolic
- Peak velocity >2.5 m/s
The American Heart Association recommends performing standard echocardiography with Doppler in all suspected cases of acute rheumatic fever, regardless of auscultation findings 2. However, the 2002 Jones Criteria Workshop reaffirmed that Doppler echocardiographic findings alone should not be classified as either a major or minor criterion without accompanying clinical context, as distinguishing physiological from pathological regurgitation remains challenging 5.
Diagnostic Algorithm
Step 1: Confirm recent Group A Streptococcal infection through throat culture, rapid antigen test, or elevated/rising antibody titers 1
Step 2: Determine population risk status (low-risk vs. moderate-to-high-risk based on local rheumatic fever incidence) 1
Step 3: Apply appropriate Jones Criteria thresholds 1:
- Low-risk populations: 2 major OR 1 major + 2 minor manifestations
- Moderate-to-high-risk populations: Same as above, but with expanded major criteria (including monoarthritis and polyarthralgia)
Step 4: Exclude alternative diagnoses such as poststreptococcal reactive arthritis, Lyme disease, serum sickness, infective endocarditis, and systemic lupus erythematosus 6
Special Scenarios and Exceptions
Recurrent Attacks
In patients with documented prior rheumatic fever or rheumatic heart disease and confirmed Group A Streptococcal infection, 2 major OR 1 major + 2 minor OR 3 minor manifestations may suffice for diagnosis 1, 4.
"Possible" Rheumatic Fever
When clinical presentation doesn't fulfill Jones Criteria but suspicion remains high, consider 12 months of secondary prophylaxis followed by reevaluation including repeat echocardiogram 1. This approach prevents progression while allowing time for diagnostic clarification 1.
Exceptions to Standard Criteria
The diagnosis may be made without strict adherence to Jones Criteria in cases of 3:
- Isolated chorea (may appear months after streptococcal infection)
- Indolent carditis (slowly progressive valve disease)
- Recurrent attacks in patients with established rheumatic heart disease
Critical Pitfalls to Avoid
Cardiac auscultation alone has very low sensitivity for diagnosing carditis and should never be relied upon as the sole method for excluding cardiac involvement. 2 Handheld echocardiography shows high sensitivity (0.87) and specificity (0.98) compared to standard echocardiography, making it a viable alternative in resource-limited settings 2.
Never diagnose acute rheumatic fever without documented streptococcal infection, except in the rare scenarios of isolated chorea or indolent carditis 2. Studies show that strict adherence to requiring documented streptococcal infection may lead to underdiagnosis, as only 29.6% of clinically confirmed cases had documented prior infection in one series 7. However, the American Heart Association maintains this requirement to prevent overdiagnosis 1.
Distinguish poststreptococcal reactive arthritis (PSRA) from acute rheumatic fever. PSRA typically develops 3-14 days after pharyngitis, has prolonged symptoms with protracted arthritis that fails to respond promptly to salicylates, and lacks the migratory pattern typical of rheumatic fever 5.
The 2002 Jones Criteria Workshop reaffirmed the validity of the major and minor Jones criteria as the accepted standard for diagnosis of initial attacks, with the consensus that no new version was justified at that time 5. However, the 2015 revision by the American Heart Association introduced the critical distinction between low-risk and moderate-to-high-risk populations and formally incorporated subclinical carditis as a major criterion 2, 1.