What is the evolution of Jones criteria for diagnosing acute rheumatic fever?

Evolution of Jones Criteria for Diagnosing Acute Rheumatic Fever

The Jones criteria for diagnosing acute rheumatic fever (ARF) have evolved significantly since their inception in 1944, with the most important update being the 2015 revision by the American Heart Association that established different diagnostic pathways based on population risk and incorporated echocardiography to detect subclinical carditis. 1, 2

Historical Development

• 1944: Original criteria established by T. Duckett Jones to aid in the clinical diagnosis of ARF
• 1992: Major revision that emphasized specificity to minimize overdiagnosis 3
• 2015: Comprehensive revision that accounted for global epidemiological changes and variability in disease presentation 1, 4

Key Changes in the 2015 Revision

Risk-Stratified Approach

The 2015 Jones criteria introduced a risk-stratified approach with different diagnostic pathways:

• Low-risk populations: ARF incidence <2 per 100,000 school-aged children or RHD prevalence ≤1 per 1000 2
• Moderate/high-risk populations: All other populations (including Turkey) 5

Major Criteria Changes

1. Carditis:

   • Subclinical carditis detected by echocardiography now qualifies as a major criterion in all populations 1, 2
   • Doppler echocardiography is essential for detecting clinical or subclinical carditis 2

2. Joint Manifestations (for moderate/high-risk populations only):

   • Added aseptic monoarthritis as a major criterion
   • Added polyarthralgia as a major criterion
   • Retained migratory polyarthritis as a major criterion 2, 5

3. Other Major Criteria (unchanged):

   • Chorea
   • Erythema marginatum
   • Subcutaneous nodules 2

Minor Criteria Changes

1. For low-risk populations:

   • Added fever ≥38.5°C as a minor criterion 5

2. For moderate/high-risk populations:

   • Monoarthralgia added as a minor criterion
   • Fever threshold lowered to ≥38°C
   • ESR threshold lowered to ≥30 mm/h 2, 5

3. For all populations (unchanged):

   • Prolonged PR interval on ECG
   • Elevated CRP ≥3.0 mg/dL 2

Diagnostic Requirements

The fundamental diagnostic approach remains consistent:

• Evidence of preceding Group A streptococcal infection PLUS either:
  • Two major criteria, OR
  • One major and two minor criteria 2, 6

Recurrent ARF Diagnosis

The 2015 revision also provided specific criteria for diagnosing recurrent ARF:

• Three minor manifestations may be sufficient for presumptive diagnosis in patients with previous ARF or RHD 1
• When only minor manifestations are present, exclusion of other more likely causes is recommended 1

"Possible" Rheumatic Fever

For cases that don't fulfill the Jones criteria but where clinical suspicion remains high:

• Consider offering 12 months of secondary prophylaxis followed by reevaluation 1
• If recurrent symptoms occur despite prophylaxis and there's no evidence of streptococcal infection or valvulitis, symptoms may not be related to ARF 1

Clinical Implications

• Echocardiography is now recommended for all patients with suspected or confirmed ARF 2, 4
• The revised criteria improve diagnosis in moderate/high-risk populations by increasing sensitivity while maintaining specificity in low-risk populations 4
• Careful differentiation of subclinical carditis from physiological valve regurgitation is essential 5
• When using joint manifestations as major criteria in moderate/high-risk populations, exclusion of other joint diseases is critical 5

Future Directions

The Jones criteria will likely continue to evolve as our understanding of ARF pathophysiology improves and new diagnostic tools emerge. Future revisions should maintain Dr. Jones' initial goal of avoiding overdiagnosis, particularly in low-risk populations 1.