<< Acute Rheumatic Fever: An Evidence-Based Approach to Diagnosis and Initial Management (Pharmacology CME)


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Evidence-based medicine requires a critical appraisal of the literature based upon study methodology and number of subjects. Not all references are equally robust. The findings of a large, prospective, randomized, and blinded trial should carry more weight than a case report.

To help the reader judge the strength of each reference, pertinent information about the study, such as the type of study and the number of patients in the study is included in bold type following the references, where available. The most informative references cited in this paper, as determined by the authors, are noted by an asterisk (*) next to the number of the reference.

  1. Zühlke LJ, Steer AC. Estimates of the global burden of rheumatic heart disease. Global Heart. 2013;8(3):189-195. (Review article)
  2. van Driel Mieke L, De Sutter An IM, Keber N, et al. Different antibiotic treatments for group A streptococcal pharyngitis. Cochrane Database of Systematic Reviews. 2013(4). (Systematic review; 17 studies)
  3. Spinks A, Glasziou PP, Del Mar CB. Antibiotics for sore throat. Cochrane Database of Systematic Reviews. 2013 Nov 5;(11):CD000023. (Systematic review; 27 studies)
  4. Altamimi S, Khalil A, Khalaiwi Khalid A, et al. Short-term late-generation antibiotics versus longer term penicillin for acute streptococcal pharyngitis in children. Cochrane Database of Systematic Reviews. 2012 Aug 15:(8) CD004872(Systematic review; 20 studies)
  5. Cilliers A, Manyemba J, Adler AJ, et al. Anti-inflammatory treatment for carditis in acute rheumatic fever. Cochrane Database Syst Rev. 2012;6:CD003176. (Systematic review; 8 randomized controlled trials, 996 patients)
  6. * National Guideline Clearinghouse. Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd edition). 2012. (National guidelines)
  7. * Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics. Circulation. 2009;119(11):1541-1551. (Scientific statement/guidelines)
  8. McDonald M, Currie BJ, Carapetis JR. Acute rheumatic fever: a chink in the chain that links the heart to the throat? Lancet Infect Dis.4(4):240-245. (Epidemiology of ARF in aboriginal communities of central and northern Australia)
  9. Bland EF. Rheumatic fever: the way it was. Circulation. 1987;76(6):1190-1195. (Historical article)
  10. Lee GM, Wessels MR. Changing epidemiology of acute rheumatic fever in the United States. Clin Infect Dis. 2006;42(4):448-450. (Editorial commentary)
  11. Kothari SS. Of history, half-truths, and rheumatic fever. Ann Pediatr Card. 2013;6(2):117-120. (Commentary)
  12. Carapetis JR, Steer AC, Mulholland EK, et al. The global burden of group A streptococcal diseases. Lancet Infect Dis. 2005;5(11):685-694. (Review article)
  13. Acute rheumatic fever and rheumatic heart disease among children — American Samoa, 2011–2012. Morbid Mortal Weekly. 2015;64(20):555-558. Available at: http://www.cdc.gov/mmwr/preview/mmwrhtml/mm6420a5.htm. Accessed December 15, 2015. (Centers for Disease Control and Prevention report)
  14. Kurahara DK, Grandinetti A, Galario J, et al. Ethnic differences for developing rheumatic fever in a low-income group living in Hawaii. Ethn Dis. 2006;16(2):357-361. (Rheumatic fever epidemiology, Hawaii)
  15. Tibazarwa KB, Volmink JA, Mayosi BM. Incidence of acute rheumatic fever in the world: a systematic review of population-based studies. Heart. 2008;94(12):1534-1540. (Systematic review; 10 studies)
  16. Miyake CY, Gauvreau K, Tani LY, et al. Characteristics of children discharged from hospitals in the United States in 2000 with the diagnosis of acute rheumatic fever. Pediatrics. 2007;120(3):503-508. (Review article)
  17. Pfoh E, Wessels MR, Goldmann D, et al. Burden and economic cost of group A streptococcal pharyngitis. Pediatrics. 2008;121(2):229-234. (Review article)
  18. Bland EF and Jones TD. Rheumatic fever and rheumatic heart disease: a twenty year report on 1000 patients followed since childhood. Circulation. 1951; 4(6): 836-843. (Clinical cases)
  19. Barash J. Rheumatic fever and post-group A streptococcal arthritis in children. Curr Infect Dis Rep. 2013;15(3):263-268. (Epidemiological and clinical data)
  20. Barash J, Mashiach E, Navon-Elkan P, et al. Differentiation of post-streptococcal reactive arthritis from acute rheumatic fever. J Pediatr. 2008;153(5):696-699. (Retrospective study; 68 patients with ARF and 159 patients with PSRA. Data were compared and analyzed with univariate, multivariate, and discriminatory analysis.)
  21. Simonini G, Taddio A, Cimaz R. No evidence yet to change American Heart Association recommendations for poststreptococcal reactive arthritis: comment on the article by van Bemmel et al. Arthritis Rheum. 2009;60(11):3516-3518; author reply 3518-3519. (Comment)
  22. Swedo SE, Leonard HL, Garvey M, et al. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases. Am J Psychiatry. 1998;155(2):264-271. (Review article; clinical cases)
  23. Williams KA, Swedo SE. Post-infectious autoimmune disorders: Sydenham’s chorea, PANDAS and beyond. Brain Res. 2015;1617:114-154. (Review article)
  24. Snider LA, Sachdev V, MaCkaronis JE, et al. Echocardiographic findings in the PANDAS subgroup. Pediatrics. 2004;114(6):e748-e751. (Prospective examination; 60 children, no control group)
  25. Snider LA, Lougee L, Slattery M, et al. Antibiotic prophylaxis with azithromycin or penicillin for childhood-onset neuropsychiatric disorders. Biol Psychiatry. 2005;57(7):788-792. (Double-blind randomized controlled trial; 23 patients)
  26. Jones T. The diagnosis of rheumatic fever. JAMA. 1944;126(8):481-484. (Review article)
  27. * Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association. Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. JAMA. 1992;268(15):2069-2073. (Clinical guidelines)
  28. * World Health Organization. Rheumatic fever and rheumatic heart disease: report of a WHO expert consultation, Geneva, 29 October-1 November 2001. 2004. (World Health Organization expert consultation)
  29. * 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;131(20):1806-1818. (Scientific statement)
  30. Burke RJ, Chang C. Diagnostic criteria of acute rheumatic fever. Autoimmun Rev. 2014;13(4-5):503-507. (Review of diagnostic criteria of ARF)
  31. Swedo SE. Sydenham’s chorea. A model for childhood autoimmune neuropsychiatric disorders. JAMA. 1994;272(22):1788-1791. (Case report)
  32. Jordan LC, Singer HS. Sydenham chorea in children. Curr Treat Options Neurol. 2003;5(4):283-290. (Review treatment options and dosing strategies)
  33. Yilmazer MM, Oner T, Tavli V, et al. Predictors of chronic valvular disease in patients with rheumatic carditis. Pediatr Cardiol. 2012;33(2):239-244. (Review article)
  34. Araujo FD, Goulart EM, Meira ZM. Prognostic value of clinical and Doppler echocardiographic findings in children and adolescents with significant rheumatic valvular disease. Ann Pediatr Cardiol. 2012;5(2):120-126. (Retrospective study; 462 patients. Multivariate logistic regression analysis used to identify factors influencing long-term heart valve disease.)
  35. Martin DR, Voss LM, Walker SJ, et al. Acute rheumatic fever in Auckland, New Zealand: spectrum of associated group A streptococci different from expected. Pediatr Infect Dis J. 1994;13(4):264-269. (Retrospective study; 2410 isolates, 32 from well-documented cases of ARF)
  36. Jaggi P. Rheumatic fever and postgroup-A streptococcal arthritis. Pediatr Infect Dis J. 2011;30(5):424-425. (Review)
  37. Mistry RM, Lennon D, Boyle MJ, et al. Septic arthritis and acute rheumatic fever in children: the diagnostic value of serological inflammatory markers. J Pediatr Orthop. 2015; 35(3):318-322. (Retrospective comparison study)
  38. Robertson KA, Volmink JA, Mayosi BM. Antibiotics for the primary prevention of acute rheumatic fever: a meta-analysis. BMC Cardiovasc Disord. 2005;5(1):11. (Meta-analysis; 10 trials, 7665 patients)
  39. American Academy of Pediatrics Committee on Infectious Diseases, Kimberlin DW, Brady MT, Jackson MA, et al. Red Book®. Report of the Committee on Infectious Diseases. Elk Grove Village, IL; 2015:737-738. (Guidelines)
  40. Thatai D, Turi ZG. Current guidelines for the treatment of patients with rheumatic fever. Drugs. 1999;57(4):545-555. (Review)
  41. Uziel Y, Hashkes PJ, Kassem E, et al. The use of naproxen in the treatment of children with rheumatic fever. J Pediatr. 2000;137(2):269-271. (Retrospective study; 19 patients)
  42. Hashkes PJ, Tauber T, Somekh E, et al. Naproxen as an alternative to aspirin for the treatment of arthritis of rheumatic fever: a randomized trial. J Pediatr. 2003;143(3):399-401. (Prospective, randomized, open-label equivalence study comparing naproxen to aspirin; 33 patients with rheumatic fever)
  43. Aron AM, Freeman JM, Carter S. The natural history of Sydenham’s chorea. Review of the literature and long-term evaluation with emphasis on cardiac ssequelae. Am J Med. 1965;38:83-95. (Review)
  44. Cardoso F. Sydenham’s chorea. Curr Treat Options Neurol. 2008;10(3):230-235. (Review)
  45. Veasy LG, Tani LY, Hill HR. Persistence of acute rheumatic fever in the intermountain area of the United States. J Pediatr. 1994;124(1):9-16. (Review article)
  46. Lennon D, Stewart J, Farrell E, et al. School-based prevention of acute rheumatic fever: a group randomized trial in New Zealand. Pediatr Infect Dis J. 2009;28(9):787-794. (Randomized controlled trial; 22,000 students)
  47. Tubridy-Clark M, Carapetis JR. Subclinical carditis in rheumatic fever: a systematic review. Int J Cardiol. 2007;119(1):54- 58. (Systematic review)
  48. Ramakrishnan S. Echocardiography in acute rheumatic fever. Ann Pediatr Cardiol. 2009;2(1):61-64. (Review)
Publication Information

Kajal Khanna, MD, JD;Deborah R. Liu, MD

Publication Date

August 2, 2016

CME Expiration Date

September 2, 2019

CME Credits

4 AMA PRA Category 1 Credits™, 4 ACEP Category I Credits, 4 AAFP Prescribed Credits, 4 AOA Category 2-A or 2-B Credits.
Specialty CME Credits: Included as part of the 4 credits, this CME activity is eligible for 2 Infectious Disease CME and 0.25 Pharmacology CME credits

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