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

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Table of Contents
 
Table of Contents
  1. Abstract
  2. Case Presentations
  3. Introduction
  4. Critical Appraisal of the Literature
  5. Epidemiology And Pathophysiology
  6. Differential Diagnosis
    1. Poststreptococcal Reactive Arthritis
    2. Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections
  7. Prehospital Care
  8. Emergency Department Evaluation
    1. The Jones Criteria
    2. International Guidelines For Application Of The Jones Criteria
    3. Clinical Features Of Major Manifestations
      1. Polyarthritis
      2. Sydenham Chorea
      3. Carditis
      4. Erythema Marginatum
      5. Subcutaneous Nodules
    4. Clinical Features Of Minor Manifestations
      1. Arthralgia
      2. Fever
      3. Elevated Acute-Phase Reactants
      4. Prolonged PR interval
    5. Evidence Of A Group A Streptococcal Infection
  9. Diagnostics Studies
    1. Laboratory Tests
    2. Imaging And Other Diagnostic Testing
  10. Treatment
    1. Antibiotic Treatment
    2. Condition-Specific Treatments
      1. Arthritis
      2. Chorea
      3. Carditis
    3. Bed Rest
    4. Surgical Management
  11. Special Populations
  12. Controversies And Cutting Edge
    1. Antibiotics For Group A Streptococcus Pharyngitis
    2. Inclusion Of Subclinical Carditis In The Modified Jones Criteria
  13. Disposition
  14. Summary
  15. Additional Resources
  16. Risk Management Pitfalls For Pediatric Patients With Acute Rheumatic Fever
  17. Time- And Cost-Effective Strategies
  18. Case Conclusions
  19. Clinical Pathway For The Diagnosis Of Patients With Suspected Acute Rheumatic Fever
  20. Tables and Figures
    1. Table 1. Differential Diagnosis Of Acute Rheumatic Fever
    2. Table 2. Comparison Of Acute Rheumatic Fever And Poststrepotoccal Reactive Arthitis
    3. Table 3. Modified Jones Criteria For The Diagnosis Of Acute Rheumatic Fever
    4. Table 4. 2002-2003 WHO Criteria For The Diagnosis Of Rheumatic Fever And Rheumatic Heart Disease (Based On The Revised Jones Criteria)
    5. Table 5. Evolution Of Diagnostic Criteria For Acute Rheumatic Fever Since 1992
    6. Table 6. Australian Guidelines For The Diagnosis Of Acute Rheumatic Fever
    7. Figure 1. Swollen And Painful Knees In A 3-Year-Old Patient With Acute Rheumatic Fever.
    8. Figure 2. Typical Appearance Of Erythema Marginatum On A Child With Acute Rheumatic Fever
    9. Figure 3. Subcutaneous Nodules Of The Elbow In A 3-Year-Old Patient With Acute Rheumatic Fever
  21. References

Abstract

Acute rheumatic fever is an inflammatory reaction involving the joints, heart, and nervous system that occurs after a group A streptococcal infection. It typically presents as a febrile illness with clinical manifestations that could include arthritis, carditis, skin lesions, or abnormal movements. Of these, the cardiac manifestations of acute rheumatic fever are most concerning, as children may present in acute heart failure and may go on to develop valvular insufficiency or stenosis. Because this is a rare presentation to emergency departments in developed countries, it is crucial for clinicians to keep a broad differential when presented with clinical presentations suspicious for acute rheumatic fever. This issue focuses on the clinical evaluation and treatment of patients with acute rheumatic fever by offering a thorough review of the literature on diagnosis and recommendations on appropriate treatment.

Case Presentations

A previously healthy 9-year-old boy who recently emigrated from Polynesia presents to the ED with joint pain and swelling. According to his parents, he has been sick for 4 days with intermittent fever and began experiencing trouble breathing over the past day. Initially, he had a painful and swollen left knee, but this seems to be better today. Upon arrival in triage, he has an axillary temperature of 39°C (102.2°F). His vital signs are as follows: heart rate, 160 beats/min; respiratory rate, 30 breaths/min; and oxygen saturation, 93% on room air. On examination, he appears tired and complains of trouble breathing when lying down. His right knee is swollen, warm, and very tender to the touch. His left ankle is also swollen and he is unable to walk because of the pain and swelling. On examination of his chest, his lung sounds are clear, but you hear a holosystolic murmur at the apex. His abdomen is soft, but you feel a liver edge 5 cm below the costal margin. The boy is alert and oriented throughout your examination. What are your first steps toward treating this child? How will you establish your diagnosis? Can this child be safely discharged from the ED? What are the potential long-term sequelae?

A 4-year-old girl is brought to the ED with a painful left knee that her parents say began hurting just last night. She has had some slight URI symptoms for the last 2 days, with a low-grade fever, mild cough, and sore throat. Her parents deny any history of trauma. During her time in the ED, she appears to have developed some swelling of her right wrist. She is afebrile in the ED with a temperature of 37.4°C (99.3°F). On your examination, she has some posterior cervical lymphadenopathy and her tonsils are erythematous and large. She has no rashes or nodules. You hear a 2/6 systolic ejection murmur and note that the swelling of her right wrist seems to have resolved. Your resident thinks that this must be a case of transient synovitis. How will you establish your diagnosis in this child?

Introduction

An emergency medicine clinician in the United States or in most developed countries is unlikely to diagnose a patient with acute rheumatic fever (ARF). Despite the low prevalence in the United States, the global burden of ARF and rheumatic heart disease remains high. It is estimated that there are 471,000 new cases of ARF each year, with the overall burden of rheumatic heart disease estimated at 15.6 million cases and over 233,000 deaths per year worldwide.1 The diagnosis of ARF relies on clinicians being aware of the diagnostic features of the disease. Since death from rheumatic heart disease is usually preventable, it is crucial to increase awareness of the symptoms of ARF.

This issue of Pediatric Emergency Medicine Practice will focus on establishing the diagnosis of ARF. The existing literature will be examined and an understanding of the clinical features of ARF and an evidence-based framework to approach the diagnosis and management of ARF will be provided.

Critical Appraisal Of The Literature

A literature review was conducted using PubMed and OVID Medline® to search for articles using the terms acute rheumatic fever, rheumatic disease, epidemiology, pharyngitis, group A streptococcal infections, subclinical carditis, rheumatic carditis, Sydenham’s chorea, chorea, and Jones criteria, with limits on all children aged 0 to 18 years. A total of 569 articles from 1964 to the present were reviewed. A search in the Cochrane Database of Systematic Reviews using the key term acute rheumatic fever yielded 4 studies.2-5 These were of limited utility and only 1 study provided some minimal guidance on the management of carditis as a result of rheumatic fever. The other reviews focused on the utility and choice of antibiotic treatment for group A streptococcal pharyngitis. A search through the National Guideline Clearinghouse database revealed an Australian guideline for prevention, diagnosis, and management of ARF and rheumatic heart disease from Rheumatic Heart Disease Australia, Menzies School of Health Research, National Heart Foundation of Australia, and the Cardiac Society of Australia and New Zealand (www.rhdaustralia.org.au/arf-rhd-guideline).6

The websites of the Canadian Pediatric Society (www.cps.ca) and the American Academy of Pediatrics (AAP) (www.aap.org) were searched for guidelines, but none were found. The AAP did, however, endorse a scientific statement from the American Heart Association (AHA) in 2009 on primary prevention of rheumatic fever that focused on group A streptococcal pharyngitis management.7 In addition to the AHA, there are also the Infectious Diseases Society of America guidelines for the management of group A Streptococcus (GAS) infections. The most detailed guidelines are from the Australian Pediatric Society. Because ARF rates remain very high in aboriginal communities in Australia, it seems prudent to include the Australian Pediatric Society recommendations in this evidence-based report.

Risk Management Pitfalls For Pediatric Patients With Acute Rheumatic Fever

Tables And Figures

Table 1. Differential Diagnosis Of Acute Rheumatic Fever

Available at: http://www.rhdaustralia.org.au/arf-rhd-guideline.

References

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)
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Publication Information
Authors

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

Publication Date

August 2, 2016

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