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The 2015 Revision of the Jones Criteria for the Diagnosis Of Review Heart Asia: first published as 10.1136/heartasia-2015-010648 on 19 August 2015. Downloaded from The 2015 revision of the Jones criteria for the diagnosis of acute rheumatic fever: implications for practice in low-income and middle-income countries Andrea Beaton,1 Jonathan Carapetis2 1Children’s National Health ABSTRACT REVISING THE STANDARD System, Cardiology, The Jones criteria has longed served as the primary In the late 19th century, improvements in housing Washington DC, USA 2Telethon Kids Institute, guideline for diagnosing acute rheumatic fever (ARF). and hygiene, which continued into the 20th University of Western Australia, However, since the first iteration in 1944, the global century and were accompanied by identification of and Princess Margaret Hospital epidemiology of ARF and our knowledge regarding the streptococcus and the advent of penicillin, led to for Children, Subiaco, Western variability of its presentation have changed. In 2015, the the virtual disappearance of ARF in the USA and Australia, Australia American Heart Association took on an ambitious and other high-income nations.4 The AHA championed Correspondence to successful revision, which accounts for these changes. the use of antibiotic prophylaxis and stewarded the Dr Jonathan Carapetis, For the first time, the criteria consider the risk within a Jones criteria, which remained an important, living Telethon Kids Institute, population and offer two separate diagnostic pathways document throughout the latter half of the 20th University of Western Australia, that prioritise specificity among those at low risk and century. The decline in ARF in the USA motivated Princess Margaret Hospital for sensitivity among those at moderate/high risk. revisions of the Jones criteria to prioritise high spe- Children, 100 Roberts Rd, 5–7 Subiaco, WA 6008, Australia; Echocardiography is now recommended in all patients cificity at the cost of lowered sensitivity. While jonathan.carapetis@ with suspected or confirmed ARF, and subclinical carditis these changes were appropriate for high-income, telethonkids.org.au can fulfil a major criterion for ARF in all populations. low-prevalence countries, concerns began to Finally, new and specific criteria are provided for the surface that the 1992 revision of the Jones criteria Accepted 5 August 2015 diagnosis of ARF recurrences. These changes improve the lacked sufficient sensitivity for use in high- – diagnosis of ARF among moderate/high-risk populations prevalence regions.8 10 and re-establish the Jones criteria as the international This sentiment was most acute in the Pacific, gold standard for ARF diagnosis. It is our hope that they where indigenous populations continue to experi- will also serve as a catalyst in the global community to ence some of the highest rates of ARF in the world. increase advocacy, improve case detection, and invest in Concerns culminated in Australia and New Zealand new research techniques that could ultimately control deciding, for the first time, to publish ARF diagnos- global ARF in our lifetimes. tic guidelines that diverged from the Jones cri- – teria,11 13 while WHO released its own version, providing guidance in areas where the Jones criteria http://heartasia.bmj.com/ were lacking, such as how to use them to diagnose 14 15 16 INTRODUCTION ARF recurrences. Other countries followed Acute rheumatic fever (ARF) killed more US youths suit, and the Jones criteria risked becoming a relic (aged 5–20 years) than any other condition during of history. the early part of the 20th century.1 The care and However, in 2012, AHA took on another revi- convalescence of patients with ARF became the sion, and made the historic decision to include focus of entire institutions, like the House of Good prominent authors living and/or working in high- Samaritan in Boston, Massachusetts. There, Dr T prevalence regions. The resulting guidelines, pub- Duckett Jones dedicated his career to the study of lished in 2015, acknowledge the importance of on September 27, 2021 by guest. Protected copyright. this devastating condition.2 The most indelible of including pretest probability in weighing sensitivity fi his contributions was the development of the first versus speci city, and include separate guidelines for clinical criteria for the diagnosis of ARF.3 These cri- low-risk (prioritising sensitivity) and moderate-to- fi 17 teria, known as the ‘Jones Criteria’ remained the high-risk (prioritising speci city) populations. benchmark for ARF diagnosis for over 50 years. This revision also embraces the improved diagnostic But much has changed over the last half-century. capacity offered by echocardiography, recommend- Development and widespread deployment of echo- ing its use in all populations, when available, for cardiography has improved our ability to diagnose the diagnosis of rheumatic carditis. In light of its and understand rheumatic carditis. The epidemi- recalibration towards the changing presentation and ology of ARF has shifted, with near-eradication in demographics of ARF, the 2015 revision will ensure most of the developed world and disease persist- the Jones criteria are re-established as the inter- ence in low-resource nations. And, study of ARF in national gold standard for ARF diagnosis. these low-resource settings has shown unaccounted- for variability in its clinical presentation. Over this 2015 JONES CRITERIA: MAJOR CHANGES AND time, the American Heart Association (AHA) has IMPLICATIONS FOR LOW-INCOME AND ’ To cite: Beaton A, published four revisions to Dr Jones criteria. The MIDDLE-INCOME COUNTRIES Carapetis J. Heart Asia latest carefully accounts for these shifts, ensuring Current diagnostic criteria 2015;7:7–11. doi:10.1136/ Dr Jones’ name and work remain relevant into the Despite over a half-century of research, no con- heartasia-2015-010648 next century. firmatory test is available for ARF, and diagnosis Beaton A, Carapetis J. Heart Asia 2015;7:7–11. doi:10.1136/heartasia-2015-010648 7 Review Heart Asia: first published as 10.1136/heartasia-2015-010648 on 19 August 2015. Downloaded from continues to rely on a constellation of clinical features. These considerations, plus evidence that strict adherence to Following the precedent set by Dr Jones, the AHA criteria cat- the 1992 Jones criteria resulted in missed ARF diagnosis,8 led egorise manifestations of ARF into major and minor criteria.3 to the development of the Australian ARF diagnostic guide- Since 1965,6 and through the 2015 revision,17 a first episode of lines.11 12 The Australian guidelines were the first to consider ARF is diagnosed when a patient has evidence of recent strepto- high-risk and low-risk populations separately, emphasising high coccal infection in addition to either two major or one major sensitivity among those at greatest risk and high specificity for and two minor criteria. It is the major and minor criteria, and those at lower risk. The 2015 Jones criteria revision has the diagnosis of recurrences, that have been the subject of embraced this risk-stratified approach. Importantly, compared ongoing debate. with the Australian guidelines which provide a definition for The 2015 updates (table 1) address three main changes in its high risk, the 2015 revision defines low risk (ARF incidence <2 diagnostic criteria: risk stratification, echocardiographic detec- per 100 000 school-aged children per year or an all-age preva- tion of subclinical carditis and joint manifestations. Each is dis- lence of RHD of ≤1 per 1000 population per year), and states cussed below, along with the rationale behind the changes. that children not clearly from a low-risk ARF population should be considered at moderate-to-high risk (moderate and high are Separation into low-risk and moderate/high-risk populations treated equally) and considered under the modified diagnostic The burden of ARF is distributed unequally around the globe. pathway.17 Practically, clinicians will likely find it easier to High-income countries have seen a near eradication of disease. decide if an individual is at low risk (based on socioeconomic Low-income/low-resource countries, or poorer populations factors) compared with high, improving the uniformity and ease within wealthy countries, have seen very little change in ARF of choosing a diagnostic pathway. incidence.18 In fact, as the majority of the world’s population lives in high-prevalence regions, ARF and rheumatic heart Addition of subclinical carditis as a major manifestation disease (RHD; ARF’s most devastating complication) remain the Clinical carditis has classically been defined as an audible leading cause of early cardiovascular morbidity and mortality. murmur consistent with aortic or mitral regurgitation.7 In 2000, Recent data estimates that at least 34.2 million people currently the AHA ARF working group acknowledged that interest was live with RHD, with over 340 000 annual deaths.19 growing in the importance of subclinical carditis, or clinically The epidemiology in high-prevalence areas also differs from silent valvular involvement only detectable through echocardi- historical ARF data. Compared with the seasonal outbreaks ography, as an indicator of ARF.22 But, it concluded that at that previously seen in industrialised countries, it is common in time there was insufficient evidence to include subclinical card- endemic regions to see hyperendemic disease patterns with itis as a major or a minor manifestation. Additionally, they high- high rates of year-round presentation. Moreover, there are data lighted that use of echocardiography to detect subclinical from high-prevalence settings such as Australia82021that the carditis could lower the specificity of the Jones criteria, and lead clinical manifestations of ARF, in particular joint presentations to overdiagnosis of ARF, and overuse of long-term penicillin and peak fever, may be substantially less dramatic, and thus prophylaxis as a result of the diagnosis.22 less clinically obvious, compared with those seen in low-risk During the past decade many studies have shown the substan- settings. tial prevalence and significance of subclinical carditis among patients with ARF, leaving little doubt as to its importance in http://heartasia.bmj.com/ ARF diagnosis.
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