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

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

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.

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