{"orpha":93568,"name":"Juvenile polymyositis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Juvenile PM"],"definition":"A rare type of juvenile idiopathic inflammatory myopathy (IIM) characterized by an onset before 18 years of age of chronic skeletal muscle inflammation, manifesting as progressive, proximal and distal muscle weakness and atrophy.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-93568/","orphanet_url":"https://www.orpha.net/en/disease/detail/93568","names":{"fr":{"name":"Polymyosite juvénile","synonyms":["PM juvénile"],"definition":"Type rare de myopathie inflammatoire idiopathique juvénile (MII), caractérisée par l'apparition avant l'âge de 18&nbsp;ans d'une inflammation chronique des muscles squelettiques, se manifestant par une faiblesse et une atrophie musculaires progressives des muscles proximaux et distaux."},"de":{"name":"Polymyositis, juvenile","definition":"Eine seltene Form der juvenilen idiopathischen entzündlichen Myopathie (IIM), die vor dem 18. Lebensjahr mit einer chronischen Skelettmuskelentzündung beginnt und sich als progressive, proximale und distale Muskelschwäche und -atrophie manifestiert."},"es":{"name":"Polimiositis juvenil","synonyms":["PM juvenil"],"definition":"Es un tipo de miopatía inflamatoria idiopática (MII) juvenil poco frecuente caracterizada por una inflamación crónica del músculo esquelético que debuta antes de los 18 años de edad, manifestándose como debilidad y atrofia progresivas de la musculatura distal y proximal."},"it":{"name":"Polimiosite giovanile","synonyms":["PM giovanile"],"definition":"Si tratta di una forma rara di miopatia infiammatoria idiopatica giovanile (IIM), che esordisce prima dei 18 anni con infiammazione cronica dei muscoli scheletrici, e si manifesta con debolezza ed atrofia progressiva dei muscoli prossimali e distali."},"nl":{"name":"Juveniele polymyositis","synonyms":["Juveniele PM"],"definition":"Een zeldzaam type van juveniele idiopathische inflammatoire myopathie (IIM), gekarakteriseerd door aanvang voor de leeftijd van 18 jaar van chronische inflammatie van skeletspieren die zich manifesteert als progressieve zwakte en atrofie van proximale en distale spieren."},"pt":{"name":"Polimiosite juvenil"},"pl":{"name":"Młodzieńcze zapalenie wielomięśniowe","synonyms":["Młodzieńcze PM"]},"cs":{"name":"Juvenilní polymyozitida","synonyms":["Juvenilní PM"]},"uk":{"name":"Ювенільний поліміозит","synonyms":["Ювенільний PM"],"definition":"A rare type of juvenile idiopathic inflammatory myopathy (IIM) characterized by an onset before 18 years of age of chronic skeletal muscle inflammation, manifesting as progressive, proximal and distal muscle weakness and atrophy."},"zh":{"name":"幼年型多发性肌炎","synonyms":["幼年型PM"],"definition":"一种罕见的青少年特发性炎症性肌病（IIM），其特征是在18岁之前开始出现慢性骨骼肌炎症，表现为进行性、近端和远端肌肉无力和萎缩。"},"tr":{"name":"Juvenil polimiyozit","synonyms":["jüvenil PM"]}},"xrefs":[{"source":"ICD-10","code":"M33.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"4A41.10","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"633330307"},{"source":"MONDO","code":"0019734","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3826988","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12742","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10076673","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":329888,"name":"Juvenile idiopathic inflammatory myopathy","classifications":[181,196]},{"orpha":567558,"name":"Non-genetic systemic disease with glomerulopathy as a major feature","classifications":[188,233]},{"orpha":280373,"name":"Rare pediatric systemic disease","classifications":[231]},{"orpha":264704,"name":"Secondary interstitial lung disease specific to childhood associated with a connective tissue disease","classifications":[184,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Childhood"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":39.0,"area":"Worldwide","status":"Validated","source":"23925923[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"23925923[PMID]_ORPHANET"}],"phenotypes":null}