Prehospital Care
Although no specific prehospital interventions exist for those with acute rheumatic fever, the patient's presentation may warrant establishment of intravenous access and placement of a cardiac monitor.
Emergency Department Care
Most patients with acute rheumatic fever (ARF) will be managed as inpatients by a multidisciplinary team of pediatricians, internists, cardiologists, infectious disease specialists, and rheumatologists. Transfer to an appropriate pediatric facility is essential. The emergency medicine physician's primary responsibilities are to suspect the diagnosis, initiate the diagnostic work-up, administer antibiotics, and treat symptoms.
Individual medications are discussed in the Medication section.
Anti-inflammatory agents are used to control the arthritis, fever, and other acute symptoms. ARF arthritis is very responsive to non-steroidal anti-inflammatory drug (NSAID) treatment. [50] Historically, high-dose aspirin was used, but naproxen and ibuprofen demonstrate similar efficacy with fewer toxic effects. [51, 52]
Sydenham chorea varies in its severity and degree of discomfort or impaired functionality. Some patients may benefit from valproic acid or carbamazepine. Those agents have similar efficacy and minimal adverse effects. For severe Sydenham chorea, corticosteroids can reduce time to remission. [2] There are also case reports showing improvement with levetiracetam, olanzapine, and risperidone. [53]
Symptoms of carditis can be managed with corticosteroids. A meta-analysis did not demonstrate clear benefit; nevertheless, it is still a consensus expert recommendation. [54, 55]
The use of intravenous immunoglobulin is not recommended, as it did not demostrate any benefit in cardiac disease at one year after treatment. [54] A small case series reported 2 cases of carditis treated with hydroxychloroquine, but the specific mechanism of action and in vivo effect is unclear. [56] Furthermore, QTc prolongation is often seen in early stages of ARF, so the use of hydroxychloroquine, which can further prolong the QTc and put the patient at risk for dysrhythmia, must be done with caution. [57]
Heart failure is managed with nitrates and diuretics, and pressors or mechanical support as needed.
Consultations
Consultations for patients with ARF are as follows:
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A pediatric consult for admission or transfer is usually required.
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A cardiology consult is essential, since carditis is a major clinical finding and also the cause of most morbidity and mortality.
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Consider consulting rheumatology, neurology, and infectious disease, especially if the diagnosis is uncertain.
Surgical Care
Although surgical intervention is rarely needed in ARF, patients who sustain significant damage and stenosis of their mitral and/or aortic valves may require valve replacement or repair at some point. As surgical techniques evolve, valve repair has become the preferred intervention. Valve replacement can be performed surgically or via catheter. [14]
Prevention
The literature reports that acute rheumatic fever (ARF) can effectively be prevented if appropriate antibiotics are given within 9 days of symptom onset. Though somewhat controversial, most authorities believe this to be a valid conclusion. Others believe that treatment of GABHS infection in most cases is not needed because most people will not progress from GAS to ARF.
At least one third of ARF episodes occur after inapparent streptococcal infections, making prevention in that group impossible. [43]
Lennon et al proposed that in New Zealand, ARF cases would decrease by 60% using a school or community clinic to treat streptococcal pharyngitis. [58]
Differences exist among nations in terms of diagnosing and treating GABHS pharyngitis. Most North American, French, and Finnish guidelines consider diagnosis of streptococcal infection essential (with either rapid antigen detection test [RADT] or formal culture) and advise antibiotic therapy when streptococci are detected. Several European guidelines consider streptococcal infection a self-limited disease and do not recommend antibiotics. The North American guidelines refer primarily to North American studies. European guidelines did not reference North American studies as frequently. [59]
Several secondary prevention antibiotic regimens exist to prevent recurrences. Duration of antibiotic prophylaxis is determined by the number of previous attacks, time since last attack, the risk of exposure to streptococcal infections, patient age, and—very importantly—presence or absence of cardiac involvement. Although the emergency medicine physician is not likely to be the prescriber of such a regimen, it is worth knowing what our colleagues may prescribe. Penicillin is still the drug of choice and may be given daily by mouth or monthly by intramuscular injection. Macrolides are acceptable in penicillin-allergic patients.
Children who have had carditis should be treated well into adulthood and may require lifelong prophylaxis. Those without carditis may be treated until they reach their 20s and after at least 5 years have elapsed since the past episode. Duration may increase if patients in this group are at risk for exposure to streptococcal infection. [24, 22]
Vaccine Development
The development of a vaccine against GAS remains a topic of intense research and clinical interest. The earliest efforts, 100 years ago, were ineffective, and vaccine trials were paused from the 1970's-2000's due to safety concerns. The past two decades of molecular research have provided new targets, [60] but a lack of financial investment hindered meaningful progress.
In 2018, the World Health Organization published the Group A Streptococcal Vaccine Research and Development Roadmap, and in 2019, a Strep A Vaccine Consortium was formed. [61] A small number of vaccine candidates are currently under development; however, only one has reached a phase II trial. No major pharmaceutical companies are working on GAS vaccines. [14, 24, 61]
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- Overview
- Presentation
- DDx
- Workup
- Treatment
- Medication
- Medication Summary
- Antimicrobials
- Penicillins, Natural
- Penicillins, Amino
- Antibiotics, Lincosamide
- Macrolides
- Cephalosporins, 1st Generation
- Glucocorticoids
- Anti-inflammatory agents
- Anticonvulsants, Other
- Diuretics
- Diuretics, Loop
- Diuretics, Potassium-Sparing
- Nitrates
- Nitrates, Angina
- Immunosuppressants
- Antipsychotics, 2nd Generation
- SV2A Ligands
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- References
