WHO guideline on the prevention and diagnosis of rheumatic fever and rheumatic heart disease

Key points

• Rheumatic fever (RF) and rheumatic heart disease (RHD) are preventable; the WHO guideline covers primary prevention, secondary prevention and treatment of RF with anti-inflammatory drugs.
• For clinically suspected group A Streptococcus (GAS) pharyngitis, WHO recommends penicillin, given intramuscularly (IM) or orally.
• IM benzathine benzylpenicillin (BPG) is the preferred first-line approach to prevent recurrence of RF in patients with prior RF or RHD; oral penicillin is an acceptable alternative.
• Children and adolescents who meet minimum criteria for RHD on echocardiography screening should receive antibiotic prophylaxis; adults aged 20 years and older may receive it.
• Penicillin allergy testing should not be used in patients with no history of penicillin allergy who are prescribed IM BPG for secondary prevention.

Overview

This World Health Organization (WHO) guideline gives evidence-informed recommendations for the prevention and diagnosis of RF and RHD, a preventable public health problem in low- and middle-income countries and in marginalized communities in middle- and high-income countries. It covers three areas: primary prevention through identifying and treating suspected GAS pharyngitis and skin infections, secondary prevention with long-term antibiotic prophylaxis, adherence support and screening for early RHD, and anti-inflammatory treatment of RF.

Disease course

RF is an autoimmune inflammatory reaction to pharyngitis, or possibly to superficial skin and skin structure infections, caused by Streptococcus pyogenes. The first episode is commonly seen in children aged 5 to 14 years; recurrences are most common within 1 year of the first episode but can occur throughout life. RHD consists of chronic structural and/or functional changes in the heart, most commonly the valves, caused by one or more episodes of RF. It usually starts in childhood, with a diagnostic peak at 20 to 39 years, and can lead to death or lifelong disability, which effective early intervention can prevent.

Primary prevention

Health workers should provide evidence-based education on the link between pharyngeal and skin infections potentially caused by GAS and RF/RHD, and on the importance of treating these infections appropriately, particularly in moderate/high-risk settings or populations (conditional recommendation, very low certainty evidence). Penicillin, IM or oral, is recommended for clinically suspected GAS pharyngitis.

Echocardiography

The guideline addresses the use of handheld echocardiography (HHE) where standard echocardiography, meaning stationary or portable fully functional devices, is not available. In populations or settings with moderate/high risk of RHD, echocardiographic screening with standard or handheld devices may be considered to improve early detection of RHD in pregnant women during antenatal care (conditional recommendation, very low certainty evidence).

Secondary prevention

IM BPG is the preferred first-line prophylaxis for patients with prior RF or RHD, and oral penicillin is acceptable when an alternative is needed. Prophylaxis should be prescribed for children and adolescents found to meet minimum criteria for RHD on echocardiography screening, to prevent disease progression, and may be prescribed for adults aged 20 years and older. The guideline also addresses interventions to improve adherence to prophylaxis.

Frequently asked questions

Which antibiotic is preferred to prevent recurrent RF?

Intramuscular benzathine benzylpenicillin; oral penicillin is an acceptable alternative.

Should children with RHD found on screening receive prophylaxis?

Yes. Children and adolescents who meet minimum echocardiographic criteria for RHD should receive antibiotic prophylaxis to prevent progression.

Is penicillin allergy testing needed before BPG?

No, not in patients without a history of penicillin allergy.

Source

World Health Organization. WHO guideline on the prevention and diagnosis of rheumatic fever and rheumatic heart disease. Geneva: World Health Organization; 29 October 2024. 53 pages. ISBN 9789240100077. Licence: CC BY-NC-SA 3.0 IGO. This summary was prepared by Medpresso from the original publication and is not a substitute for the full text or medical advice.