title: Acute rheumatic fever and rheumatic heart disease.
abstract: Acute rheumatic fever (ARF) is the result of an autoimmune response to pharyngitis caused by infection with group A Streptococcus. The long-term damage to cardiac valves caused by ARF, which can result from a single severe episode or from multiple recurrent episodes of the illness, is known as rheumatic heart disease (RHD) and is a notable cause of morbidity and mortality in resource-poor settings around the world. Although our understanding of disease pathogenesis has advanced in recent years, this has not led to dramatic improvements in diagnostic approaches, which are still reliant on clinical features using the Jones Criteria, or treatment practices. Indeed, penicillin has been the mainstay of treatment for decades and there is no other treatment that has been proven to alter the likelihood or the severity of RHD after an episode of ARF. Recent advances - including the use of echocardiographic diagnosis in those with ARF and in screening for early detection of RHD, progress in developing group A streptococcal vaccines and an increased focus on the lived experience of those with RHD and the need to improve quality of life - give cause for optimism that progress will be made in coming years against this neglected disease that affects populations around the world, but is a particular issue for those living in poverty.
authors.affiliation: Telethon Kids Institute, the University of Western Australia, PO Box 855, West Perth, Western Australia 6872, Australia.|Princess Margaret Hospital for Children, Perth, Western Australia, Australia.
authors.forename: Jonathan R
authors.initials: JR
authors.lastname: Carapetis
authors.affiliation: Children's National Health System, Washington, District of Columbia, USA.
authors.forename: Andrea
authors.initials: A
authors.lastname: Beaton
authors.affiliation: Department of Microbiology and Immunology, Biomedical Research Center, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA.
authors.forename: Madeleine W
authors.initials: MW
authors.lastname: Cunningham
authors.affiliation: Heart Institute (InCor), University of São Paulo, School of Medicine, São Paulo, Brazil.|Institute for Immunology Investigation, National Institute for Science and Technology, São Paulo, Brazil.
authors.forename: Luiza
authors.initials: L
authors.lastname: Guilherme
authors.affiliation: Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Ganesan
authors.initials: G
authors.lastname: Karthikeyan
authors.affiliation: Department of Medicine, Groote Schuur Hospital, University of Cape Town, Cape Town, South Africa.
authors.forename: Bongani M
authors.initials: BM
authors.lastname: Mayosi
authors.affiliation: Children's National Health System, Washington, District of Columbia, USA.
authors.forename: Craig
authors.initials: C
authors.lastname: Sable
authors.affiliation: Department of Paediatrics, the University of Melbourne, Melbourne, Victoria, Australia.|Murdoch Childrens Research Institute, Melbourne, Victoria, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Steer
authors.affiliation: Green Lane Paediatric and Congenital Cardiac Services, Starship Hospital, Auckland, New Zealand.|Department of Paediatrics, University of Auckland, Auckland, New Zealand.
authors.forename: Nigel
authors.initials: N
authors.lastname: Wilson
authors.affiliation: Telethon Kids Institute, the University of Western Australia, PO Box 855, West Perth, Western Australia 6872, Australia.
authors.forename: Rosemary
authors.initials: R
authors.lastname: Wyber
authors.affiliation: Department of Medicine, Groote Schuur Hospital, University of Cape Town, Cape Town, South Africa.|Department of Paediatric Cardiology, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa.
authors.forename: Liesl
authors.initials: L
authors.lastname: Zühlke
mesh_terms: D000328:Adult; D000900:Anti-Bacterial Agents / Q000494:pharmacology / Q000627:therapeutic use; D015551:Autoimmunity; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010406:Penicillins / Q000494:pharmacology / Q000627:therapeutic use; D010612:Pharyngitis / Q000209:etiology; D011322:Primary Prevention / Q000379:methods; D012213:Rheumatic Fever / Q000150:complications* / Q000276:immunology; D012214:Rheumatic Heart Disease / Q000209:etiology* / Q000401:mortality; D055502:Secondary Prevention / Q000379:methods; D013290:Streptococcal Infections / Q000150:complications* / Q000503:physiopathology; D013297:Streptococcus pyogenes / Q000187:drug effects / Q000472:pathogenicity
chemical_list: D000900:Anti-Bacterial Agents; D010406:Penicillins