Emergent resuscitation in acute rheumatic fever is not well defined in the literature. Suggested urgent actions on initial presentation include:

1. 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.

Major Criteria:

• 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.

Minor Criteria:

• 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.

Investigations:

1. Echocardiography with Doppler in all confirmed and suspected cases of ARF (Class 1, Level B).
2. Anti-Streptolysin O titers or other streptococcal antibodies (e.g. anti-DNAse B) can help bolster diagnosis of GAS pharyngitis (Class 1, Level B).
3. GAS throat culture or positive rapid GAS antigen test in the case of high pre-test probability for GAS pharyngitis (Class 1, Level B).
4. CRP and ESR.

Figure 1: Erythema Marginatum. https://commons.wikimedia.org/wiki/File:Erythema_marginatum.jpg.  Image accessed from Wikimedia Commons.

Figure 2: Scarlet Fever Rash. https://commons.wikimedia.org/wiki/File:Scarlet_fever_2.jpg

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.

Symptomatic Treatment:

• 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.

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.

Patients would require transfer to a capable centre facilitate the above investigations (e.g., echocardiogram, blood tests) and in-patient pediatric care.

Patients with acute rheumatic fever, and especially those with carditis require referral to cardiology for ongoing management and serial echocardiography.

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3. 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.

    

   
   

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