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Last reviewed: 16 Mar 2025
Last updated: 10 Apr 2024

Summary

Definition

History and exam

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

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

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

Treatment algorithm

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

SC

Disclosures

AS declares that she has no competing interests.

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