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Rheumatic fever (RF) is a disease diagnosed using the Jones criteria. The criteria were initially published in 1944 and have been revised a number of times, the last revision being in 2002 by the American Heart Association.1,2 A case is presented here that highlights the limitations of the Jones criteria in high prevalence areas such as New Zealand.Case reportA 6-year-old New Zealand-born Samoan boy presented with a 2-month history of migratory joint pain in the knees, ankles and wrists. His parents also reported that he had a sore throat 1-month prior. He had also experienced leg stiffness in the mornings. There was a history of possible fever the night before presentation without a history of trauma, rash or joint swelling. The arthralgia was treated with paracetamol syrup. No non-steroidal anti-inflammatory drugs were taken. The patients mother and two siblings had RF as children.On examination he was afebrile with normal vital signs. All joints were non-tender with normal ranges of motion and the absence of clinical effusions. His cardiac examination revealed a 2/6 ejection systolic murmur loudest at the left sternal edge. He had bilateral cervical lymphadenopathy. The examination was otherwise normal.Laboratory investigations revealed elevated antistreptococcal antibody titres; anti-streptolysin O titre (ASOT) was 711 IU/mL (normal <400) and anti-DNAse B was 1230 U/mL (normal <680). Erythrocyte sedimentation rate (ESR) was 95mm/hr. An electrocardiogram showed a PR interval of 0.2s (prolonged for his age). The transthoracic echocardiogram was normal.Serological tests were performed to assess for alternative causes of arthralgia including Cytomegalovirus, Rubella, Epstein-Barr virus, Parvovirus, Mycoplasma, Yersinia and hepatitis; these were all negative.A working diagnosis of RF was made and he was started on benzathine benzylpenicillin. He was followed up at the Rheumatic Fever Outpatient Clinic at 4 weeks where he was found to be symptom free. The transthoracic echocardiogram was once again normal and the ECG showed the PR interval to be 0.14s. The ESR was 17, ASOT 634 and Anti-DNAse 800.DiscussionUsing the Jones criteria this boy would not have been diagnosed with RF, as he had no major manifestations (see Figure 1). He did however have three confirmed minor manifestations: elevated ESR; prolonged PR interval; arthralgia and laboratory evidence of streptococcus infection.The consulting paediatrician and infectious diseases specialist came to a clinical decision to treat this boy for RF given the high prevalence observed in Mori and Pacific peoples, his family history and the lack of an alternative explanation for his recent illness.Less convincingly, the observed reduction in the PR interval and ESR at 4 weeks suggested that this was an acute event. Furthermore, the alternative causes of arthralgia, mainly viral infections, were excluded with serology as detailed above. Figure 1. Jones criteria2 Note: Diagnosis of RF requires: two major manifestations or one major and two minor manifestations. The prevalence of RF in New Zealand Mori and Pacific peoples is approximately 40 to 100 cases per 100,000 per year, compared to less than 10 per 100,000 per year in European New Zealanders.3 It has been shown that the strict application of the Jones criteria in areas of high prevalence, such as Australias Northern Territory, will result in a substantial number of RF cases being missed. 4 Another study in the Northern Territory found that 25% of Aboriginal patients diagnosed with RF with the main symptom being arthralgia, had no clinical evidence of arthritis. 5 The authors went on to propose that polyarthralgia should be made a major criterion for probable RF.5 Without a gold standard test for RF diagnosis, clinicians practising in New Zealand have reason to consider broadening the diagnostic criteria when assessing people of Maori and Pacific descent given that the Jones criteria are based on USA data where RF prevalence is low. Broadening the criteria would increase the sensitivity and decrease the specificity of the criteria. This would result in a greater number of patients being identified as having RF and therefore receiving treatment for the condition, thus decreasing the risk of cardiac complications. However, it would also mean a greater number of patients without RF would be exposed to unnecessary penicillin treatment for a number of years. The National Heart Foundation of New Zealand and the Cardiac Society of Australia and New Zealand have advocated broadening the criteria for populations with high prevalence but have not remarked on polyarthralgia specifically.6 Larger studies in high prevalence areas are needed to assess the adequacy of the Jones criteria in these populations.

Summary

Abstract

Rheumatic fever is a disease diagnosed using the Jones criteria. The Jones criteria were designed using data from areas with a low prevalence of rheumatic fever. In New Zealand there is a high prevalence of rheumatic fever amongst Maori and Pacific peoples. A case is presented where a child of Samoan ethnicity is diagnosed and treated for rheumatic fever without fulfilling the Jones criteria. Evidence supporting the broadening of the diagnostic criteria in high prevalence areas is highlighted.

Aim

Method

Results

Conclusion

Author Information

Nikola Lilic, Otorhinolaryngology Registrar, Department of ORL, Head and Neck Surgery, Middlemore Hospital, Auckland and Clinical Medical Education Fellow, University of Auckland; Priyanka R Kumar, General Medicine Registrar, Department of General Medicine, North Shore Hospital, Auckland

Acknowledgements

Correspondence

Nikola Lilic, 85 Park Rd, University of Auckland, Grafton, Auckland 1023, New Zealand. Fax: +64 (0)9 3737555

Correspondence Email

Nikola.z.lilic@gmail.com

Competing Interests

Jones, TD. The diagnosis of rheumatic fever. JAMA. 1944;126:481.Ferrieri P, Jones Criteria Working Group. Proceedings of the Jones Criteria workshop. Circulation. 2002;106(19):2521.Lennon D. Rheumatic fever, a preventable disease? The New Zealand experience. In: Martin DR, Tagg JR, eds. Streptococci and streptococcal diseases: entering the new millennium. Porirua: Institute of Environmental Science and Research. 2000, p503-12.Carapetis JR, Currie BJ. Rheumatic fever in a high incidence population: the importance of monoarthritis and low grade fever. Arch Dis Child. 2001;85(3):223.Ralph A, Jacups S, McGough K, et al. The Challenge of acute rheumatic fever diagnosis in a high-incidence population: A prospective study and proposed guidelines for diagnosis in Australias Northern Territory. Heart Lung Circ. 2006;15:113-118.Atatoa-Carr P, Lennon D, Wilson N, et al. Rheumatic fever diagnosis, management, and secondary prevention: a New Zealand guideline. N Z Med J. 2008;121(1271):59-69. http://journal.nzma.org.nz/journal/121-1271/2975/content.pdf

For the PDF of this article,
contact nzmj@nzma.org.nz

View Article PDF

Rheumatic fever (RF) is a disease diagnosed using the Jones criteria. The criteria were initially published in 1944 and have been revised a number of times, the last revision being in 2002 by the American Heart Association.1,2 A case is presented here that highlights the limitations of the Jones criteria in high prevalence areas such as New Zealand.Case reportA 6-year-old New Zealand-born Samoan boy presented with a 2-month history of migratory joint pain in the knees, ankles and wrists. His parents also reported that he had a sore throat 1-month prior. He had also experienced leg stiffness in the mornings. There was a history of possible fever the night before presentation without a history of trauma, rash or joint swelling. The arthralgia was treated with paracetamol syrup. No non-steroidal anti-inflammatory drugs were taken. The patients mother and two siblings had RF as children.On examination he was afebrile with normal vital signs. All joints were non-tender with normal ranges of motion and the absence of clinical effusions. His cardiac examination revealed a 2/6 ejection systolic murmur loudest at the left sternal edge. He had bilateral cervical lymphadenopathy. The examination was otherwise normal.Laboratory investigations revealed elevated antistreptococcal antibody titres; anti-streptolysin O titre (ASOT) was 711 IU/mL (normal <400) and anti-DNAse B was 1230 U/mL (normal <680). Erythrocyte sedimentation rate (ESR) was 95mm/hr. An electrocardiogram showed a PR interval of 0.2s (prolonged for his age). The transthoracic echocardiogram was normal.Serological tests were performed to assess for alternative causes of arthralgia including Cytomegalovirus, Rubella, Epstein-Barr virus, Parvovirus, Mycoplasma, Yersinia and hepatitis; these were all negative.A working diagnosis of RF was made and he was started on benzathine benzylpenicillin. He was followed up at the Rheumatic Fever Outpatient Clinic at 4 weeks where he was found to be symptom free. The transthoracic echocardiogram was once again normal and the ECG showed the PR interval to be 0.14s. The ESR was 17, ASOT 634 and Anti-DNAse 800.DiscussionUsing the Jones criteria this boy would not have been diagnosed with RF, as he had no major manifestations (see Figure 1). He did however have three confirmed minor manifestations: elevated ESR; prolonged PR interval; arthralgia and laboratory evidence of streptococcus infection.The consulting paediatrician and infectious diseases specialist came to a clinical decision to treat this boy for RF given the high prevalence observed in Mori and Pacific peoples, his family history and the lack of an alternative explanation for his recent illness.Less convincingly, the observed reduction in the PR interval and ESR at 4 weeks suggested that this was an acute event. Furthermore, the alternative causes of arthralgia, mainly viral infections, were excluded with serology as detailed above. Figure 1. Jones criteria2 Note: Diagnosis of RF requires: two major manifestations or one major and two minor manifestations. The prevalence of RF in New Zealand Mori and Pacific peoples is approximately 40 to 100 cases per 100,000 per year, compared to less than 10 per 100,000 per year in European New Zealanders.3 It has been shown that the strict application of the Jones criteria in areas of high prevalence, such as Australias Northern Territory, will result in a substantial number of RF cases being missed. 4 Another study in the Northern Territory found that 25% of Aboriginal patients diagnosed with RF with the main symptom being arthralgia, had no clinical evidence of arthritis. 5 The authors went on to propose that polyarthralgia should be made a major criterion for probable RF.5 Without a gold standard test for RF diagnosis, clinicians practising in New Zealand have reason to consider broadening the diagnostic criteria when assessing people of Maori and Pacific descent given that the Jones criteria are based on USA data where RF prevalence is low. Broadening the criteria would increase the sensitivity and decrease the specificity of the criteria. This would result in a greater number of patients being identified as having RF and therefore receiving treatment for the condition, thus decreasing the risk of cardiac complications. However, it would also mean a greater number of patients without RF would be exposed to unnecessary penicillin treatment for a number of years. The National Heart Foundation of New Zealand and the Cardiac Society of Australia and New Zealand have advocated broadening the criteria for populations with high prevalence but have not remarked on polyarthralgia specifically.6 Larger studies in high prevalence areas are needed to assess the adequacy of the Jones criteria in these populations.

Summary

Abstract

Rheumatic fever is a disease diagnosed using the Jones criteria. The Jones criteria were designed using data from areas with a low prevalence of rheumatic fever. In New Zealand there is a high prevalence of rheumatic fever amongst Maori and Pacific peoples. A case is presented where a child of Samoan ethnicity is diagnosed and treated for rheumatic fever without fulfilling the Jones criteria. Evidence supporting the broadening of the diagnostic criteria in high prevalence areas is highlighted.

Aim

Method

Results

Conclusion

Author Information

Nikola Lilic, Otorhinolaryngology Registrar, Department of ORL, Head and Neck Surgery, Middlemore Hospital, Auckland and Clinical Medical Education Fellow, University of Auckland; Priyanka R Kumar, General Medicine Registrar, Department of General Medicine, North Shore Hospital, Auckland

Acknowledgements

Correspondence

Nikola Lilic, 85 Park Rd, University of Auckland, Grafton, Auckland 1023, New Zealand. Fax: +64 (0)9 3737555

Correspondence Email

Nikola.z.lilic@gmail.com

Competing Interests

Jones, TD. The diagnosis of rheumatic fever. JAMA. 1944;126:481.Ferrieri P, Jones Criteria Working Group. Proceedings of the Jones Criteria workshop. Circulation. 2002;106(19):2521.Lennon D. Rheumatic fever, a preventable disease? The New Zealand experience. In: Martin DR, Tagg JR, eds. Streptococci and streptococcal diseases: entering the new millennium. Porirua: Institute of Environmental Science and Research. 2000, p503-12.Carapetis JR, Currie BJ. Rheumatic fever in a high incidence population: the importance of monoarthritis and low grade fever. Arch Dis Child. 2001;85(3):223.Ralph A, Jacups S, McGough K, et al. The Challenge of acute rheumatic fever diagnosis in a high-incidence population: A prospective study and proposed guidelines for diagnosis in Australias Northern Territory. Heart Lung Circ. 2006;15:113-118.Atatoa-Carr P, Lennon D, Wilson N, et al. Rheumatic fever diagnosis, management, and secondary prevention: a New Zealand guideline. N Z Med J. 2008;121(1271):59-69. http://journal.nzma.org.nz/journal/121-1271/2975/content.pdf

For the PDF of this article,
contact nzmj@nzma.org.nz

View Article PDF

Rheumatic fever (RF) is a disease diagnosed using the Jones criteria. The criteria were initially published in 1944 and have been revised a number of times, the last revision being in 2002 by the American Heart Association.1,2 A case is presented here that highlights the limitations of the Jones criteria in high prevalence areas such as New Zealand.Case reportA 6-year-old New Zealand-born Samoan boy presented with a 2-month history of migratory joint pain in the knees, ankles and wrists. His parents also reported that he had a sore throat 1-month prior. He had also experienced leg stiffness in the mornings. There was a history of possible fever the night before presentation without a history of trauma, rash or joint swelling. The arthralgia was treated with paracetamol syrup. No non-steroidal anti-inflammatory drugs were taken. The patients mother and two siblings had RF as children.On examination he was afebrile with normal vital signs. All joints were non-tender with normal ranges of motion and the absence of clinical effusions. His cardiac examination revealed a 2/6 ejection systolic murmur loudest at the left sternal edge. He had bilateral cervical lymphadenopathy. The examination was otherwise normal.Laboratory investigations revealed elevated antistreptococcal antibody titres; anti-streptolysin O titre (ASOT) was 711 IU/mL (normal <400) and anti-DNAse B was 1230 U/mL (normal <680). Erythrocyte sedimentation rate (ESR) was 95mm/hr. An electrocardiogram showed a PR interval of 0.2s (prolonged for his age). The transthoracic echocardiogram was normal.Serological tests were performed to assess for alternative causes of arthralgia including Cytomegalovirus, Rubella, Epstein-Barr virus, Parvovirus, Mycoplasma, Yersinia and hepatitis; these were all negative.A working diagnosis of RF was made and he was started on benzathine benzylpenicillin. He was followed up at the Rheumatic Fever Outpatient Clinic at 4 weeks where he was found to be symptom free. The transthoracic echocardiogram was once again normal and the ECG showed the PR interval to be 0.14s. The ESR was 17, ASOT 634 and Anti-DNAse 800.DiscussionUsing the Jones criteria this boy would not have been diagnosed with RF, as he had no major manifestations (see Figure 1). He did however have three confirmed minor manifestations: elevated ESR; prolonged PR interval; arthralgia and laboratory evidence of streptococcus infection.The consulting paediatrician and infectious diseases specialist came to a clinical decision to treat this boy for RF given the high prevalence observed in Mori and Pacific peoples, his family history and the lack of an alternative explanation for his recent illness.Less convincingly, the observed reduction in the PR interval and ESR at 4 weeks suggested that this was an acute event. Furthermore, the alternative causes of arthralgia, mainly viral infections, were excluded with serology as detailed above. Figure 1. Jones criteria2 Note: Diagnosis of RF requires: two major manifestations or one major and two minor manifestations. The prevalence of RF in New Zealand Mori and Pacific peoples is approximately 40 to 100 cases per 100,000 per year, compared to less than 10 per 100,000 per year in European New Zealanders.3 It has been shown that the strict application of the Jones criteria in areas of high prevalence, such as Australias Northern Territory, will result in a substantial number of RF cases being missed. 4 Another study in the Northern Territory found that 25% of Aboriginal patients diagnosed with RF with the main symptom being arthralgia, had no clinical evidence of arthritis. 5 The authors went on to propose that polyarthralgia should be made a major criterion for probable RF.5 Without a gold standard test for RF diagnosis, clinicians practising in New Zealand have reason to consider broadening the diagnostic criteria when assessing people of Maori and Pacific descent given that the Jones criteria are based on USA data where RF prevalence is low. Broadening the criteria would increase the sensitivity and decrease the specificity of the criteria. This would result in a greater number of patients being identified as having RF and therefore receiving treatment for the condition, thus decreasing the risk of cardiac complications. However, it would also mean a greater number of patients without RF would be exposed to unnecessary penicillin treatment for a number of years. The National Heart Foundation of New Zealand and the Cardiac Society of Australia and New Zealand have advocated broadening the criteria for populations with high prevalence but have not remarked on polyarthralgia specifically.6 Larger studies in high prevalence areas are needed to assess the adequacy of the Jones criteria in these populations.

Summary

Abstract

Rheumatic fever is a disease diagnosed using the Jones criteria. The Jones criteria were designed using data from areas with a low prevalence of rheumatic fever. In New Zealand there is a high prevalence of rheumatic fever amongst Maori and Pacific peoples. A case is presented where a child of Samoan ethnicity is diagnosed and treated for rheumatic fever without fulfilling the Jones criteria. Evidence supporting the broadening of the diagnostic criteria in high prevalence areas is highlighted.

Aim

Method

Results

Conclusion

Author Information

Nikola Lilic, Otorhinolaryngology Registrar, Department of ORL, Head and Neck Surgery, Middlemore Hospital, Auckland and Clinical Medical Education Fellow, University of Auckland; Priyanka R Kumar, General Medicine Registrar, Department of General Medicine, North Shore Hospital, Auckland

Acknowledgements

Correspondence

Nikola Lilic, 85 Park Rd, University of Auckland, Grafton, Auckland 1023, New Zealand. Fax: +64 (0)9 3737555

Correspondence Email

Nikola.z.lilic@gmail.com

Competing Interests

Jones, TD. The diagnosis of rheumatic fever. JAMA. 1944;126:481.Ferrieri P, Jones Criteria Working Group. Proceedings of the Jones Criteria workshop. Circulation. 2002;106(19):2521.Lennon D. Rheumatic fever, a preventable disease? The New Zealand experience. In: Martin DR, Tagg JR, eds. Streptococci and streptococcal diseases: entering the new millennium. Porirua: Institute of Environmental Science and Research. 2000, p503-12.Carapetis JR, Currie BJ. Rheumatic fever in a high incidence population: the importance of monoarthritis and low grade fever. Arch Dis Child. 2001;85(3):223.Ralph A, Jacups S, McGough K, et al. The Challenge of acute rheumatic fever diagnosis in a high-incidence population: A prospective study and proposed guidelines for diagnosis in Australias Northern Territory. Heart Lung Circ. 2006;15:113-118.Atatoa-Carr P, Lennon D, Wilson N, et al. Rheumatic fever diagnosis, management, and secondary prevention: a New Zealand guideline. N Z Med J. 2008;121(1271):59-69. http://journal.nzma.org.nz/journal/121-1271/2975/content.pdf

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