Acute Rheumatic Fever
Cardiovascular, Infections, Pediatrics
First 5 Minutes
Emergent resuscitation in acute rheumatic fever is not well defined in the literature. Suggested urgent actions on initial presentation include:
- Address any treat heart failure, if present.
Acute rheumatic fever (ARF) is an autoimmune inflammatory reaction that may occur secondary to group A streptococcal (GAS) pharyngitis if left untreated. ARF, especially if recurrent, may lead to the development of rheumatic heart disease (RHD).
RHD is preventable through the detection and treatment of GAS pharyngitis insofar as avoiding episodes of ARF.
GAS pharyngitis occurs primarily in children between the ages of 5 and 15 in the winter and early spring.
Rheumatic heart disease secondary to acute rheumatic fever remains a serious problem in low and middle income countries and among marginalized populations around the world. Rheumatic heart disease is an important cause of cardiovascular morbidity and mortality.
Diagnosis is clinical and based on the presence of various major and/or minor features. The features required to diagnose ARF depend on whether the presentation is new or recurrent.
Patients are divided into low-risk (incidence of ARF ≤2 per 100,000 children or RHD ≤1 per 1,000 total population) and moderate- and high-risk populations.
Patients in Canada would be classified as low risk (2.9 cases per 1,000,000 as per Canadian Pediatric Surveillance data 2004 to 2007). An exception is among populations with inadequate or crowded housing and lower socioeconomic status. A 2015 study found the incidence of ARF among First Nation children in NW Ontario was 21.3 per 100,000 (moderate- to high-risk group).
Initial ARF → evidence of preceding GAS infection and 2 MAJOR manifestations OR 1 MAJOR and 2 MINOR manifestations.
Recurrent ARF → evidence of preceding GAS infection and 2 MAJOR manifestations OR 1 MAJOR and 2 MINOR manifestations OR 3 MINOR manifestations.
- Low-Risk Pop: clinical and/or subclinical carditis (including echocardiographic valvulitis), polyarthritis, chorea, erythema marginatum, and subcutaneous nodules.
- Moderate- to High-Risk Pop: clinical and/or subclinical carditis, monoarthritis/polyarthritis/polyathralgia (polyarthralgia after exclusion of other causes), chorea, erythema marginatum, and subcutaneous nodules.
- Low-Risk Pop: polyarthralgia, fever >38.5 degrees, ESR ≥60mm in first hour and/or CRP ≥3.0mg/dL, and prolonged PR interval (after accounting for age variability unless carditis is a major criterion).
- Moderate- to High-Risk Pop: monoarthralgia, fever >38.5 degrees, ESR ≥30mm in first hour and/or CRP ≥3.0mg/dL, and prolonged PR interval (after accounting for age variability unless carditis is a major criterion).
Summary of ARF Clinical Findings:
- Carditis (50-70%) and arthritis of large joints (35-66%) are the most common manifestations.
- Sydenham chorea presents as involuntary and non-stereotypical movements of the trunk or extremities.
- Arthritis involves the large joints and lasts approximately 4-weeks.
- Erythema marginatum (<6%) presents as an erythematous, evanescent, non-pruritic, and serpiginous rash that typically presents on the trunk and proximal extremities.
- Subcutaneous nodules (0-10%) present on extensor surfaces.
GAS Features: Sudden onset sore throat, pain with swallowing, fever, scarlet fever rash, headache, red and swollen uvula, tender anterior lymphadenopathy, patient 5 – 15 years old, tonsillopharyngeal erythema and exudates, nausea, vomiting, and abdominal pain.
- Echocardiography with Doppler in all confirmed and suspected cases of ARF (Class 1, Level B).
- Anti-Streptolysin O titers or other streptococcal antibodies (e.g. anti-DNAse B) can help bolster diagnosis of GAS pharyngitis (Class 1, Level B).
- GAS throat culture or positive rapid GAS antigen test in the case of high pre-test probability for GAS pharyngitis (Class 1, Level B).
- CRP and ESR.
Treatment is divided into primary prevention (treatment) and secondary prevention.
For primary treatment of acute rheumatic fever, the underlying GAS pharyngitis is treated with penicillin V 250mg BID for children and 500mg BID for adolescents or those ≥27kg. Alternatively, benzathine penicillin G 600,000 IU for children and 1.2M IU for adolescents and those ≥27kg.
For secondary prevention, benzathine penicillin G every 4 weeks in doses of 600,000IU for children and 1.2M IU for adolescents and those ≥27kg is used. If oral is preferred, patients can take penicillin V 0.5g daily for children and 1g daily for adolescents and those ≥27kg.
Duration of treatment is based on expert opinion.
Patients with carditis causing heart failure or rheumatic heart disease will require additional cardiac treatment which will not be discussed in this clinical summary.
- Arthritis should be treated with acetaminophen until the diagnosis is established, then treatment with naproxen 10-20mg/kg/day BID can be initiated.
- Treatment of chorea is supportive.
- Aspirin and corticosteroids have been shown not to demonstrate improvement in cardiac outcomes at one year after diagnosis.
Criteria For Hospital Admission
Most patients with acute rheumatic fever are admitted to hospital to facilitate diagnostic work-up, initiate treatment, and provide education to the patient and the family.
Criteria For Transfer To Another Facility
Patients would require transfer to a capable centre facilitate the above investigations (e.g., echocardiogram, blood tests) and in-patient pediatric care.
Criteria For Close Observation And/or Consult
Patients with acute rheumatic fever, and especially those with carditis require referral to cardiology for ongoing management and serial echocardiography.
Quality Of Evidence?
We are highly confident that the true effect lies close to that of the estimate of the effect. There is a wide range of studies included in the analyses with no major limitations, there is little variation between studies, and the summary estimate has a narrow confidence interval.
We consider that the true effect is likely to be close to the estimate of the effect, but there is a possibility that it is substantially different. There are only a few studies and some have limitations but not major flaws, there are some variations between studies, or the confidence interval of the summary estimate is wide.
When the true effect may be substantially different from the estimate of the effect. The studies have major flaws, there is important variations between studies, of the confidence interval of the summary estimate is very wide.
Information presented in this clinical summary is primarily taken from the American Heart Association and is endorsed by the American Academy of Pediatrics. The quality of evidence varies throughout the review, but is consistently rated as “Class I” and “Grade A or B” (data derived from multiple high-quality clinical trials or single high-quality randomized/nonrandomized studies).
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Gerber MA, Baltimore RS, Eaton CB, Gewitz M, Rowley AH, Shulman ST, Taubert KA. Prevention of Rheumatic Fever and Diagnosis and Treatment of Acute Streptococcal Pharyngitis. Circulation. 2009; 119: 1541-1551. DOI: 10.1161/CIRCULATIONAHA.109.191959
Gordon J, Kirlew M, Schreiber Y, Saginur R, Bocking N, Blakelock B, Haavaldsrud M, Kennedy C, Farrel T, Douglas L, Kelly L. Acute rheumatic fever in First Nations communities in Northwestern Ontario. Canadian Family Physician. 2015; 61: 881-886. PMID: 26759842; PMCID: PMC4607335.
Gewitz MH, Baltimore RS, Tani LY, Sable CA, Shulman ST, Carapetis J, Remenyi B, Taubert KA, Bolger AF, Beerman L, Mayosi BM, Beaton A, Pandian NG, Kaplan EL. Revision of the Jones Criteria for the Diagnosis of Acute Rheumatic Fever in the Era of Doppler Echocardiography. Circulation. 2015;131:1806-1818. DOI: 10.1161/CIR.0000000000000205
The purpose of this document is to provide health care professionals with key facts and recommendations for the diagnosis and treatment of patients in the emergency department. This summary was produced by Emergency Care BC (formerly the BC Emergency Medicine Network) and uses the best available knowledge at the time of publication. However, healthcare professionals should continue to use their own judgment and take into consideration context, resources and other relevant factors. Emergency Care BC is not liable for any damages, claims, liabilities, costs or obligations arising from the use of this document including loss or damages arising from any claims made by a third party. Emergency Care BC also assumes no responsibility or liability for changes made to this document without its consent.
Last Updated Dec 27, 2022
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