What are the Jones criteria for diagnosing rheumatic fever?

Jones Criteria for Diagnosing Rheumatic Fever

The Jones criteria, first established in 1944 by T. Duckett Jones and revised multiple times by the American Heart Association, remain the gold standard for diagnosing acute rheumatic fever, requiring evidence of preceding Group A Streptococcal infection plus either two major manifestations or one major and two minor manifestations. 1, 2

Historical Development

The Jones criteria have evolved significantly since their original publication:

• 1944: First published by T. Duckett Jones
• Multiple revisions by the American Heart Association
• 1992: Updated to focus on initial attacks of acute rheumatic fever
• 2015: Most recent revision incorporating echocardiography and risk stratification

Current Jones Criteria (2015 Revision)

Major Manifestations

• Carditis (clinical or subclinical)
• Polyarthritis
• Chorea
• Erythema marginatum
• Subcutaneous nodules

Minor Manifestations

• Fever (≥38.5°C for low-risk populations, ≥38°C for moderate/high-risk populations)
• Arthralgia
• Elevated acute phase reactants:
  • ESR ≥60 mm/h for low-risk populations, ≥30 mm/h for moderate/high-risk populations
  • CRP ≥3.0 mg/dL
• Prolonged PR interval on ECG (age-adjusted)

Evidence of Preceding Group A Streptococcal Infection

• Positive throat culture for Group A Streptococcus
• Positive rapid antigen detection test
• Elevated or rising anti-streptolysin O titer
• Elevated anti-DNase B

Diagnostic Algorithm

1. For initial diagnosis of ARF:

   • Two major criteria, OR
   • One major and two minor criteria
   • PLUS evidence of preceding Group A Streptococcal infection 1, 2

2. For recurrent attacks in patients with established rheumatic heart disease:

   • Two major, OR
   • One major and two minor, OR
   • Three minor criteria
   • PLUS evidence of preceding Group A Streptococcal infection 1

3. Risk stratification approach (2015 revision):

   • Different diagnostic thresholds apply to low-risk, moderate-risk, and high-risk populations 1
   • Subclinical carditis detected by echocardiography is now considered a major criterion 1

Role of Echocardiography

The 2015 revision significantly expanded the role of echocardiography:

• Doppler echocardiography is now essential for documenting clinical or subclinical carditis 1, 2
• Detects valvular regurgitation (particularly mitral and aortic) even in the absence of audible murmurs
• Helps evaluate:
  • Valve morphology and function
  • Chamber size and ventricular function
  • Presence and size of pericardial effusions 1

Important Clinical Considerations

• Mitral insufficiency is the most common echocardiographic finding (detected in up to 93% of cases) 3
• Arthritis is more frequent than arthralgia, though no specific pattern of joint involvement is consistently observed 3
• Chorea may present with normal inflammatory markers, requiring different diagnostic considerations 2

Potential Pitfalls

• Strict adherence to Jones criteria requiring documented Group A Streptococcal infection may lead to underdiagnosis, particularly in resource-limited settings 3
• Misinterpreting elevated ASO titers as indicating acute rather than recent past infection can lead to unnecessary antibiotic use 2
• Echocardiography without accompanying auscultatory findings was previously insufficient to be the sole criterion for valvulitis, though this has changed with the 2015 revision 4, 1

The Jones criteria continue to evolve as our understanding of rheumatic fever advances, with the most significant recent changes being the incorporation of echocardiography and risk stratification based on population prevalence of rheumatic fever.