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?
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.
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.
Available at: http://www.rhdaustralia.org.au/arf-rhd-guideline.
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.
Kajal Khanna, MD, JD;Deborah R. Liu, MD
August 2, 2016
September 2, 2019
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
Upon completion of this article, you should be able to:
Date of Original Release: August 1, 2016. Date of most recent review: July15, 2016. Termination date: August 1, 2019.
Accreditation: EB Medicine is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians. This activity has been planned and implemented in accordance with the Essential Areas and Policies of the ACCME.
Credit Designation: EB Medicine designates this enduring material for a maximum of 4 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
ACEP Accreditation: Pediatric Emergency Medicine Practice is also approved by the American College of Emergency Physicians for 48 hours of ACEP Category I credit per annual subscription.
AAP Accreditation: This continuing medical education activity has been reviewed by the American Academy of Pediatrics and is acceptable for a maximum of 48 AAP credits per year. These credits can be applied toward the AAP CME/CPD Award available to Fellows and Candidate Fellows of the American Academy of Pediatrics.
AOA Accreditation: Pediatric Emergency Medicine Practice is eligible for up to 48 American Osteopathic Association Category 2A or 2B credit hours per year.
Other Specialty CME: Included as part of the 4 credits, this CME activity is eligible for 2 Infectious Disease CME and 0.25 Pharmacology CME credits, subject to your state and institutional requirements.
Needs Assessment: The need for this educational activity was determined by a survey of medical staff, including the editorial board of this publication; review of morbidity and mortality data from the CDC, AHA, NCHS, and ACEP; and evaluation of prior activities for emergency physicians.
Target Audience: This enduring material is designed for emergency medicine physicians, physician assistants, nurse practitioners, and residents.
Goals: Upon completion of this activity, you should be able to: (1) demonstrate medical decision-making based on the strongest clinical evidence; (2) cost-effectively diagnose and treat the most critical presentations; and (3) describe the most common medicolegal pitfalls for each topic covered.
Discussion of Investigational Information: As part of the journal, faculty may be presenting investigational information about pharmaceutical products that is outside Food and Drug Administration–approved labeling. Information presented as part of this activity is intended solely as continuing medical education and is not intended to promote off-label use of any pharmaceutical product.
Faculty Disclosure: It is the policy of EB Medicine to ensure objectivity, balance, independence, transparency, and scientific rigor in all CME-sponsored educational activities. All faculty participating in the planning or implementation of a sponsored activity are expected to disclose to the audience any relevant financial relationships and to assist in resolving any conflict of interest that may arise from the relationship. In compliance with all ACCME Essentials, Standards, and Guidelines, all faculty for this CME activity were asked to complete a full disclosure statement. The information received is as follows: Dr. Khanna, Dr. Liu, Dr. Hollander, Dr. Leibovich, Dr. Vella, Dr. Wang, Dr.Damilini, and their related parties report no significant financial interest or other relationship with the manufacturer(s) of any commercial product(s) discussed in this educational presentation.
Commercial Support: This issue of Emergency Medicine Practice did not receive any commercial support.
Earning Credit: Two Convenient Methods: (1) Go online to www.ebmedicine.net/CME and click on the title of the article. (2) Mail or fax the CME Answer And Evaluation Form (included with your June and December issues) to EB Medicine.
Hardware/Software Requirements: You will need a Macintosh or PC to access the online archived articles and CME testing.
Additional Policies: For additional policies, including our statement of conflict of interest, source of funding, statement of informed consent, and statement of human and animal rights, visit www.ebmedicine.net/policies.