Prevention and Diagnosis of Rheumatic Fever and Rheumatic Heart Disease

Publication Date: October 29, 2024
Last Updated: October 29, 2024

Summary of Recommendations

Health education

WHO recommends that health workers provide evidence-based education focused on the relationship between infections of the pharynx and skin potentially caused by group A Streptococcus (GAS), and rheumatic fever/rheumatic heart disease (RF/RHD), and thus the importance of treating these infections appropriately, particularly in moderate/high risk settings or populations. (C, VL )
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Group A streptococcal pharyngitis

WHO, with guidance from the Guideline Development Group (GDG), was unable to make a recommendation at this time on clinical prediction rules (CPRs) or on other sets of signs and symptoms that have sufficient diagnostic accuracy for use in children, adolescents or adults who present with sore throat. (, )
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Children, adolescents and adults with sore throat and a positive diagnostic test (either point-of-care (POC) testing or microbial confirmation) for GAS pharyngitis should be treated with antibiotics to prevent RF/RHD. (S, M )
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In populations at moderate to high risk of RF and RHD and where diagnostic testing to confirm GAS (with either POC testing or microbial confirmation) is not available, children and adolescents with clinically-suspected GAS pharyngitis should be treated with antibiotics to prevent RF/RHD. (S, VL )
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For patients with a positive diagnostic test for GAS pharyngitis or with clinically-suspected GAS, WHO recommends penicillin (intramuscular (IM) or oral) as first-line treatment for the prevention of RF/RHD. (C, L )
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Skin and skin structure infections

WHO, with guidance from the GDG, was unable to make a recommendation at this time either for or against any specific CPR to be used when GAS skin infection is suspected. (, )
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WHO, with guidance from the GDG, was unable to make a recommendation at this time either for or against antibiotic treatment of skin and skin structure infection(s) (SSSIs), whether laboratory confirmed or clinically diagnosed, for the specific purpose of preventing RF or RHD. (, )
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Diagnosis of rheumatic fever

The Jones criteria should be used for RF diagnosis in children, adolescents and adults with suspected RF. (S, L )
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Echocardiography in the diagnosis of rheumatic fever and rheumatic heart disease

Among children, adolescents and adults with suspected RF or RHD in settings where standard echocardiography is not available, handheld echocardiography (HHE) can be used for diagnosis of RF-carditis and RHD. (Strong recommendation, very low certainty evidence for RF-carditis, moderate certainty for RHD) (S, VL )
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In populations or settings with moderate/high risk of RHD, echocardiographic screening using standard or handheld devices may be considered, to improve early detection of RHD among pregnant women during antenatal care. (C, VL )
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In populations with moderate/high RHD prevalence, echocardiographic screening using standard echocardiography or HHE may be implemented for early detection of RHD among children and adolescents 5 to 19 years of age. (S, H )
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Antibiotic prophylaxis for the prevention of recurrent rheumatic fever

Children, adolescents and adults diagnosed with RF or RHD should be prescribed antibiotic prophylaxis to prevent RF recurrence. (S, M )
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Antibiotic prophylaxis should be prescribed for children and adolescents found to meet minimum criteria for RHD on echocardiography screening to prevent disease progression. (S, M )
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Antibiotic prophylaxis may be prescribed for adults 20 years of age and older found to meet minimum criteria for RHD on echocardiography screening. (C, VL )
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Antibiotic prophylaxis should be given to children and adolescents who have advanced RHD to prevent RF recurrence. (S, VL )
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Antibiotic prophylaxis can be given to adults 20 years of age and older who have advanced RHD to prevent RF recurrence based on shared decision-making between the patient and treating health care provider. (C, VL )
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IM benzathine benzylpenicillin (BPG), is the preferred first-line approach to prevent recurrence of RF in patients with prior RF or RHD. (S, M )
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If an alternative to IM BPG is needed (recommendation 4), oral penicillin is acceptable for RF and RHD prophylaxis. (C, M )
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Penicillin allergy testing should not be used in patients who have no history of penicillin allergy and who are prescribed IM BPG for secondary prevention of RHD. (Good practice statement) (U, U)
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An oral penicillin test dose may be given prior to IM BPG administration for patients who have a history of mild penicillin allergy; that is, in patients without a prior history of anaphylaxis, angioedema, StevenJohnson’s syndrome or toxic epidermal necrolysis. (Conditional recommendation, low certainty evidence for immediate allergy and anaphylaxis; very low for delayed allergy)

(C, VL )
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A local anaesthetic may be added to the injectable solution to reduce injection pain in patients who receive IM BPG for secondary prevention of RHD. (C, L )
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Patients who are prescribed antibiotics for secondary prophylaxis of RF or RHD should be supported to improve treatment adherence. (S, L )
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Anti-inflammatory agents for the treatment of rheumatic fever

The GDG was unable to formulate a recommendation. Thus, WHO does not recommend either for or against the use of anti-inflammatory agents for children, adolescents and adults diagnosed with RF to prevent the progression to RHD. These agents include aspirin, non-steroidal anti-inflammatory drugs (NSAIDs), intravenous immunoglobulin and corticosteroids. (, )
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Recommendation Grading

Disclaimer

The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.

Overview

Title

Prevention and Diagnosis of Rheumatic Fever and Rheumatic Heart Disease

Authoring Organization

World Health Organization

Publication Month/Year

October 29, 2024

Last Updated Month/Year

October 29, 2024

Document Type

Guideline

Country of Publication

Global

Document Objectives

Rheumatic fever (RF) and Rheumatic heart disease (RHD) are a preventable public health problem in low- and middle-income countries and in marginalized communities in middle- and high-income countries. RF is an autoimmune inflammatory reaction to throat infections (pharyngitis) or possibly to superficial skin and skin structure infections caused by Streptococcus pyogenes, a group A beta‐haemolytic Streptococcus (GAS) bacterium. The first episode of RF is commonly seen in children aged 5 to 14 years. Recurrent episodes are most common within 1 year of the first episode but can occur throughout the life course. RHD is characterized by chronic structural and/or functional changes in the heart, most commonly in the valves, caused by one or more episodes of rheumatic fever (RF). RHD most commonly starts in childhood with a diagnostic peak in young adults  aged 20 to 39 years. RHD can lead to death or lifelong disability, however, effective early intervention can prevent premature morbidity and mortality. The WHO guideline on the prevention and diagnosis of rheumatic fever (RF) and rheumatic heart disease (RHD) provides evidence-informed recommendations for the prevention and management of RF and RHD. It encompasses three areas; 1) primary prevention of rheumatic fever and rheumatic heart disease, specifically the identification and treatment of suspected group A (beta-haemolytic) Streptococcus (GAS) pharyngitis and skin infections; 2) secondary prevention of recurrent rheumatic fever and of rheumatic heart disease, specifically use of long-term antibiotic prophylaxis, interventions to increase adherence to antibiotic prophylaxis, and screening for early rheumatic heart disease; and 3) management of rheumatic fever, specifically the treatment with anti-inflammatory drugs.

Inclusion Criteria

Male, Female, Adolescent, Adult

Health Care Settings

Ambulatory, Outpatient

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Diagnosis, Assessment and screening, Treatment, Management

Diseases/Conditions (MeSH)

D012213 - Rheumatic Fever, D012214 - Rheumatic Heart Disease

Keywords

rheumatic heart disease, rheumatic fever

Source Citation

WHO guideline on the prevention and diagnosis of rheumatic fever and rheumatic heart disease. Geneva: World Health Organization; 2024. Licence: CC BY-NC-SA 3.0 IGO.