Rheumatic fever

Last reviewed: 22 Feb 2023

Last updated: 18 Nov 2022

Acute rheumatic fever (ARF) continues to cause a large burden of mortality and morbidity in low- and middle-income countries. It is less common in high-income countries, but continues to be seen in indigenous communities and during outbreaks.

Currently, no single test can diagnose ARF. Diagnosis is clinical and relies on the Jones criteria.

The 5 major manifestations of ARF are carditis, arthritis, chorea, erythema marginatum, and subcutaneous nodules, of which the most common are carditis and arthritis.

The Jones criteria were revised in 2015 to include separate criteria for low-risk and moderate- to high-risk populations and introduce the use of Doppler echocardiography to diagnose subclinical carditis.

While all other manifestations of ARF resolve without sequelae, carditis can lead to chronic rheumatic heart disease (RHD).

Secondary prophylaxis is thought to ameliorate the progression of ARF to chronic RHD, but even the mildest forms of carditis can result in chronic RHD and decreased survival.

The prognosis of established rheumatic valvular disease remains guarded, especially with severe disease.

Definition

Acute rheumatic fever (ARF) is an autoimmune disease that mostly occurs following group A streptococcal throat infection, although there is growing evidence of ARF following skin infection in certain regions of the world. It can affect multiple systems, including the joints, heart, brain, and skin. Only the effects on the heart can lead to permanent illness; chronic changes to the heart valves are referred to as chronic rheumatic heart disease (RHD). Without long-term penicillin secondary prophylaxis, ARF can recur, leading to cumulative damage to the cardiac valvular tissue.

History and exam

Key diagnostic factors

fever
joint pain

More key diagnostic factors

Other diagnostic factors

recent sore throat or scarlet fever
recent skin infection
chest pain
shortness of breath
palpitations
heart murmur
pericardial rub
signs of cardiac failure
swollen joints
restlessness
clumsiness
emotional lability and personality changes
jerky, uncoordinated choreiform movements
inability to maintain protrusion of the tongue
milkmaid's grip
spooning sign
pronator sign
erythema marginatum
subcutaneous nodules
pregnancy or taking oral contraceptive pill

Other diagnostic factors

Risk factors

poverty
overcrowded living quarters
family history of rheumatic fever
HLA association
genetic susceptibility
indigenous populations; Aboriginal Australian, Asian, and Pacific Islanders
D8/17 B cell antigen positivity

More risk factors

Diagnostic investigations

1st investigations to order

erythrocyte sedimentation rate (ESR)
CRP
WBC count
blood cultures
electrocardiogram
chest x-ray
echocardiogram
throat culture
rapid antigen test for group A streptococci
anti-streptococcal serology
rapid molecular test

More 1st investigations to order

Treatment algorithm

INITIAL

monoarthritis in unconfirmed rheumatic fever

ACUTE

possible rheumatic fever

confirmed rheumatic fever

ONGOING

all patients following acute treatment

Contributors

Authors

Liesl Zühlke, MBChB DCH FCPaeds Cert Card (Paeds) MPH FACC FESC MSc PhD

Vice-President South African Medical Research Council - Extramural Research and Internal Portfolio

Director Children's Heart Disease Research Unit

Paediatric Cardiologist, Division of Paediatric Cardiology, Department of Paediatrics

Red Cross Children's Hospital

Cape Heart Institute and Institute of Infectious Diseases and Molecular Medicine

Faculty of Health Sciences

University of Cape Town

Cape Town

South Africa

Disclosures

LZ has been funded by the South African Medical Research Council, NRF, and through the African Research Leader award jointly by the UK Medical Research Council (MRC) and the UK Department for International Development (DFID) under the MRC/DFID Concordat agreement. LZ is a board member of the World Heart Federation, the NCD Alliance, and FoodForward South Africa. LZ an author of the UpToDate rheumatic heart disease topic. None of the above are competing interests.

John Lawrenson, null

Head of Clinical Unit

Paediatric Cardiology Service of the Western Cape

Red Cross Children's and Tygerberg Hospital

Stellenbosch University and University of Cape Town

Cape Town

South Africa

Disclosures

JL declares that he has no competing interests.

Acknowledgements

Professor Liesl Zühlke and Professor John Lawrenson would like to gratefully acknowledge Dr Rachel Webb, Dr Andrew C. Steer, and Dr Jonathan Carapetis, previous contributors to this topic.

Disclosures

RW declares that she has no competing interests; she is an active researcher and clinician in acute rheumatic fever/rheumatic heart disease and is a co-investigator on a (non-industry) grant funded by the Health Research Council of New Zealand and gives educational talks and has prepared manuscripts on rheumatic fever solely in capacity as a University of Auckland academic and Paediatric Infectious Diseases Specialist. ACS and JC declare that they have no competing interests.

Peer reviewers

Salah Zaher, MD

Professor of Pediatrics

Division of Pediatric Cardiology

Faculty of Medicine

University of Alexandria

Cardiologist

El Shatby Children's Hospital

Alexandria

Egypt

Disclosures

SZ declares that she has no competing interests.

Nigel Wilson, FRACP

Paediatric Cardiologist/Interventional Cardiologist

Paediatric and Congenital Cardiac Services

Green Lane Clinical Services

Starship Children's Hospital

Auckland

New Zealand

Disclosures

NW declares that he has no competing interests.

Andrea Summer, MD

Assistant Professor of Pediatrics

Medical University of South Carolina

Charleston

Disclosures

AS declares that she has no competing interests.

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Guidelines
- Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease
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Rheumatic fever

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic investigations

1st investigations to order

Treatment algorithm

monoarthritis in unconfirmed rheumatic fever

possible rheumatic fever

confirmed rheumatic fever

all patients following acute treatment

Contributors

Authors

Liesl Zühlke, MBChB DCH FCPaeds Cert Card (Paeds) MPH FACC FESC MSc PhD

Disclosures

John Lawrenson, null

Disclosures

Acknowledgements

Disclosures

Peer reviewers

Salah Zaher, MD

Disclosures

Nigel Wilson, FRACP

Disclosures

Andrea Summer, MD

Disclosures

Differentials

Guidelines

Patient leaflets

Videos

Log in or subscribe to access all of BMJ Best Practice

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