Jones criteria for diagnosis of acute rheumatic fever

Clinical background

Acute rheumatic fever (ARF) has no single test that can make the diagnosis. The combination of a variable clinical picture, manifestations that overlap with other inflammatory conditions, and the fact that up to a third of patients do not report a preceding sore throat makes clinical assessment difficult [2]. The Jones criteria, originally produced in 1944 and revised by the American Heart Association (AHA) in 1956, 1965, 1984 and 1992, have long been the international standard tool for structuring the diagnosis [1].

The most recent major revision was published in 2015 and was the first since 1992 [1]. It was driven by three problems: the global epidemiology had shifted so that ARF is largely a disease of low- and middle-income countries, echocardiography had become available worldwide and proved able to detect cardiac involvement even without auscultatory findings, and the 1992 criteria were too rigid in applying the same rules across populations with very different disease burdens [3]. The revision now stratifies the criteria by population risk and incorporates Doppler echocardiography as a full diagnostic tool.

Applying the Jones criteria

The diagnosis follows two pathways depending on whether this is a first episode or a recurrence:

First episode of ARF: two major criteria, or one major criterion plus two minor criteria, combined with evidence of a preceding group A streptococcal (GAS) infection.

$$\text{Diagnosis of a first episode of ARF} = \begin{cases} 2,\text{major} \ \text{or} \ 1,\text{major} + 2,\text{minor} \end{cases} + \text{evidence of GAS}$$

Recurrent ARF in a patient with known previous ARF or rheumatic heart disease: the same combinations as above, or three minor manifestations alone. Evidence of GAS is not required for the recurrence pathway with three minor criteria if such evidence cannot be obtained.

$$\text{Diagnosis of recurrent ARF} = \begin{cases} 2,\text{major} + \text{GAS} \ 1,\text{major} + 2,\text{minor} + \text{GAS} \ 3,\text{minor (no GAS requirement)} \end{cases}$$

The criteria are stratified by population risk category. Low risk is defined as populations in which the ARF incidence is $\leq 2/100,000$ school-aged children or the RHD prevalence is $\leq 1/1,000$ across all ages. All other populations are classified as moderate/high risk [1]. The stratification affects the thresholds and definitions of several manifestations, which is a central difference from the 1992 criteria.

Major criteria (unchanged between the risk groups except for arthritis):

• Carditis, clinical and/or subclinical on echocardiography. Subclinical carditis is defined as echocardiographically demonstrated valvular involvement without auscultatory findings and now counts as a full major criterion in both risk groups [1, 3].
• Arthritis: at low risk, polyarthritis is required. At moderate/high risk, monoarthritis, polyarthritis or polyarthralgia suffices.
• Sydenham chorea
• Erythema marginatum
• Subcutaneous nodules

Minor criteria (thresholds vary with risk group):

• Arthralgia: monoarthralgia at moderate/high risk, polyarthralgia at low risk. It cannot be counted if arthritis has already been used as a major criterion.
• Fever: $\geq 38{.}0,^\circ\text{C}$ at moderate/high risk, $\geq 38{.}5,^\circ\text{C}$ at low risk.
• Raised ESR ($\geq 30$ at moderate/high risk, $\geq 60$ at low risk) and/or CRP $\geq 3{.}0,\text{mg/dL}$.
• Prolonged PR interval for age, if carditis is not already counted as a major criterion.

The underlying basis is the AHA expert committee, which conducted a systematic review of the published studies on Doppler echocardiography in ARF and on population-dependent differences in clinical presentation [1]. It is not a classical prediction model derived from a single cohort, but a consensus-based classification using the ACC/AHA system of class of recommendation and level of evidence.

Interpretation in practice

The calculator gives a binary diagnosis, not a risk percentage. The point of the criteria is that they are structured so that the different risk groups optimise for different goals: in low-risk populations, specificity is prioritised (avoiding overdiagnosis); in moderate/high-risk populations, sensitivity is prioritised (not missing cases) [3].

Echocardiography is recommended for all patients with suspected or confirmed ARF, whether or not auscultatory findings are present [1, 3]. A patient with normal auscultation but echocardiographically demonstrated valvular regurgitation meets the major criterion of carditis.

Validation and performance

Since the Jones criteria are a consensus-based classification and not a statistical model, traditional measures such as a c-statistic and calibration are lacking from the derivation. There are, however, systematic reviews comparing the diagnostic accuracy of the criteria with simplified algorithms and against the outcome of rheumatic heart disease (RHD).

A systematic review from 2025 identified only three studies (four reports) that met the inclusion criteria for assessing the diagnostic accuracy of simplified algorithms compared with the modified Jones criteria [2]. A complete work-up with all laboratory tests and echocardiography, corresponding to a national referral hospital, showed an AUC of 0.91 with a sensitivity of 84% and a specificity of 87%. When the modified Jones criteria were used without echocardiography, sensitivity fell to 79% with specificity remaining high (100%, AUC 0.90). Simplified algorithms using clinical data alone performed considerably worse (AUC 0.69, sensitivity 66%, specificity 68%) [2].

An important finding was that 2.5 to 5% of children and young adults in high-prevalence areas who did not meet the full modified Jones criteria nonetheless developed RHD during follow-up [2]. This indicates that the criteria, even in their most complete form, do not capture everyone who later develops cardiac damage, particularly in endemic populations.

The review also highlights that access to echocardiography is the single most important factor for diagnostic performance. Without echocardiography, subclinical carditis goes unnoticed and sensitivity falls appreciably [2].

Limitations

Application to populations: the criteria are designed for a global context in which the epidemiology of ARF varies dramatically. In low-risk populations, which include most of Europe and North America, the thresholds are higher (a stricter fever requirement, higher ESR limits, a requirement for polyarthritis) in order to minimise false-positive diagnoses [1, 3]. This is rational from a public health perspective but may mean that occasional genuine cases in low-risk populations are missed, particularly if the patient presents atypically or if evidence of GAS is hard to obtain.

Dependence on echocardiography: the fact that subclinical carditis now counts as a major criterion is a strength where echocardiography is available, but a problem where it is not. In resource-limited settings, where ARF is commonest, access to echocardiography is often lacking, which leads to systematic underdiagnosis [2, 3].

Evidence of GAS: the requirement for evidence of a preceding GAS infection can be difficult to meet. Throat culture has a substantial false-negative rate, and antibody titres may be rising without reaching a diagnostic level at the time of assessment [2]. For recurrent ARF the calculator applies a relaxation: three minor manifestations can diagnose a recurrence without evidence of GAS, an important difference from a first episode.

Manifestations cannot be counted twice: a given manifestation cannot count as both major and minor. Arthritis as a major criterion excludes arthralgia as a minor criterion in the same patient. In the same way, carditis as a major criterion excludes a prolonged PR interval as a minor criterion. This is a common source of error in clinical use.

Chorea as an exception: Sydenham chorea may be the only manifestation of ARF and may appear months after the triggering infection, at which point evidence of GAS may be negative. The AHA criteria in practice allow chorea alone to make the diagnosis, which the calculator's strict formula does not fully capture.

Limited evidence base: the systematic review found only three studies with usable data on diagnostic accuracy [2]. The evidence base for the performance of the criteria is therefore thin, and most of the recommendations rest on expert consensus and indirect evidence rather than on prospective validation studies.

Applying the low-risk thresholds

Most high-income settings fall by definition into the low-risk population for ARF, with an incidence well below the threshold of $\leq 2/100,000$ school-aged children. This means that the stricter thresholds for low-risk populations apply: fever $\geq 38{.}5,^\circ\text{C}$, ESR $\geq 60$, polyarthritis as a major criterion and polyarthralgia as a minor criterion. In such settings, ARF is an uncommon diagnosis, and suspicion should be raised above all in children with a recent sore throat and arthritis or carditis. In 2024, the WHO published a global guideline on the prevention and diagnosis of rheumatic fever and rheumatic heart disease, which builds on and endorses the 2015 Jones criteria as the diagnostic framework [4].

References

1. Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation 2015. PMID: 25908771
2. Providencia R, Aali G, Zhu F, et al. Diagnostic test accuracy of simplified algorithms for diagnosing acute rheumatic fever: a systematic review. Communications Medicine 2025. PMID: 40796655
3. Beaton A, Carapetis J. The 2015 revision of the Jones criteria for the diagnosis of acute rheumatic fever: implications for practice in low-income and middle-income countries. Heart Asia 2015. PMID: 27326214
4. World Health Organization. WHO guideline on the prevention and diagnosis of rheumatic fever and rheumatic heart disease. Geneva: WHO 2024. PMID: 39631006