{"orpha":3099,"name":"Rheumatic fever","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Acute rheumatic fever"],"definition":"Rheumatic fever (RF) is a multisystem inflammatory disease occurring as a post-infectious, nonsuppurative sequela of untreated streptococcus pyogenes (Group A streptococcus [GAS]) pharyngitis, and mainly occurs in individuals aged 5 to 15 years. The most common presenting signs are fever, migratory polyarthritis and carditis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-3099/","orphanet_url":"https://www.orpha.net/en/disease/detail/3099","names":{"fr":{"name":"Rhumatisme articulaire aigu","definition":"Le rhumatisme articulaire aigu (RAA) est une maladie inflammatoire multi-systémique. Il s'agit d'une complication non suppurée d'une pharyngite non traitée, due au <i>Streptococcus pyogenes</i> (streptocoque groupe A [SGA]). Le RAA affecte surtout les individus âgés de 5 à 15 ans. Les manifestations les plus fréquents sont une fièvre, une polyarthrite migratrice et une cardite."},"de":{"name":"Rheumatisches Fieber","synonyms":["Akutes rheumatisches Fieber"],"definition":"Das Rheumatische Fieber (RF) ist eine entzündliche Multisystem-Erkrankung. Sie ist die postinfektiöse, nicht-eitrige Folge einer unbehandelten <i>Streptococcus pyogenes</i> (Gruppe A- Streptokokken [GAS])-Pharyngitis und tritt hauptsächlich bei Personen im Alter zwischen 5 und 15 Jahren auf. Die häufigsten Krankheitsanzeichen sind Fieber, migratorische Polyarthritis und Karditis."},"es":{"name":"Fiebre reumática","synonyms":["Fiebre reumática aguda"],"definition":"La fiebre reumática (FR) es una enfermedad inflamatoria multisistémica que se produce como secuela post-infecciosa no supurativa de una faringitis por streptococcus pyogenes sin tratar (estreptococo del grupo A [GAS]). Ocurre principalmente en individuos de 5 a 15 años. Los signos de presentación más comunes son fiebre, poliartritis migratoria y carditis."},"it":{"name":"Febbre reumatica","synonyms":["Febbre reumatica acuta"],"definition":"La febbre reumatica (RF) è una malattia infiammatoria multisistemica, che si manifesta come sequela non suppurativa post-infettiva della faringite non trattata da Streptococcus pyogenes (streptococco di gruppo A [GAS]), per lo più in soggetti tra i 5 e i 15 anni. Può interessare il tessuto connettivo del cuore, delle articolazioni, della cute e dei vasi. I segni di esordio più comuni sono la febbre, la poliartrite migrante e la cardite."},"nl":{"name":"Reumatische koorts","synonyms":["Acute reumatische koorts"],"definition":"Reumatische koorts (RF) is een inflammatoire multisysteemziekte die voorkomt als post-infectueuze, niet-etterende restletsels van onbehandelde Streptococcus pyogenes (Groep A Streptococcus [GAS])-faryngitis (keelontsteking), en voornamelijk voorkomt bij individuen met een leeftijd van 5 tot 15 jaar. De meest voorkomende symptomen zijn koorts, migrerende polyartritis en carditis."},"pt":{"name":"Reumatismo articular agudo","synonyms":["Febre reumática"]},"pl":{"name":"Gorączka reumatyczna","synonyms":["Ostra gorączka reumatyczna"],"definition":"Gorączka reumatyczna (RF) to wieloukładowa choroba zapalna występująca jako poinfekcyjne, nieropne następstwo nieleczonego zapalenia gardła wywołanego przez Streptococcus pyogenes (paciorkowce grupy A), dotycząca głównie osób w przedziale wiekowym 5-15 lat. Najczęstszymi objawami są gorączka, wędrujące zapalenie wielostawowe i zapalenie mięśnia sercowego."},"cs":{"name":"Revmatická horečka","synonyms":["Akutní revmatická horečka"]},"uk":{"name":"Ревматична лихоманка","synonyms":["Гостра ревматична лихоманка"],"definition":"Rheumatic fever (RF) is a multisystem inflammatory disease occurring as a post-infectious, nonsuppurative sequela of untreated streptococcus pyogenes (Group A streptococcus [GAS]) pharyngitis, and mainly occurs in individuals aged 5 to 15 years. The most common presenting signs are fever, migratory polyarthritis and carditis."},"zh":{"name":"风湿热","synonyms":["急性风湿热"]},"tr":{"name":"Romatizmal ateş","synonyms":["Akut romatizmal ateş"]}},"xrefs":[{"source":"OMIM","code":"268240","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"I00","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"I01.0","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"I01.1","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"I01.2","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"I01.8","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"I01.9","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"1B40","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"1B41","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"MONDO","code":"0017767","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D012213","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0035436","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"5699","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10039054","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":182222,"name":"Rare systemic disease","classifications":[196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":5.0,"area":"Europe","status":"Validated","source":"21386976[PMID]_[EXPERT]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-5 / 10 000","value":12.5,"area":"Canada","status":"Validated","source":"21386976[PMID]_[EXPERT]_ ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-5 / 10 000","value":12.5,"area":"Spain","status":"Validated","source":"21386976[PMID]_[EXPERT]_ ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":">1 / 1000","value":100.0,"area":"Australia","status":"Validated","source":"21386976[PMID]_[EXPERT]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-5 / 10 000","value":30.0,"area":"India","status":"Validated","source":"21386976[PMID]_[EXPERT]_ ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":">1 / 1000","value":100.0,"area":"Mexico","status":"Validated","source":"21386976[PMID]_[EXPERT]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":5.0,"area":"Latin America","status":"Validated","source":"21386976[PMID]_[EXPERT]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":7.6,"area":"New Zealand","status":"Validated","source":"23254495[PMID]_21386976[PMID]_ [EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0002039","label":"Anorexia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001369","label":"Arthritis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012733","label":"Macule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001482","label":"Subcutaneous nodule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001646","label":"Abnormal aortic valve morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001633","label":"Abnormal mitral valve morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011675","label":"Arrhythmia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100749","label":"Chest pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002072","label":"Chorea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100584","label":"Endocarditis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012819","label":"Myocarditis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000980","label":"Pallor","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100776","label":"Recurrent pharyngitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000246","label":"Sinusitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001654","label":"Abnormal heart valve morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002167","label":"Abnormality of speech or vocalization","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002103","label":"Abnormality of the pleura","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010318","label":"Aplasia/Hypoplasia of the abdominal wall musculature","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000708","label":"Atypical behavior","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002019","label":"Constipation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010526","label":"Dysgraphia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010522","label":"Dyslexia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000421","label":"Epistaxis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002380","label":"Fasciculations","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001288","label":"Gait disturbance","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100248","label":"Hemiballismus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002076","label":"Migraine","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000100","label":"Nephrotic syndrome","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001701","label":"Pericarditis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002093","label":"Respiratory insufficiency","frequency":"Occasional (29-5%)","criteria":null}]}}