{"orpha":103919,"name":"Autoimmune pancreatitis","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["AIP"],"definition":"A rare pancreatic disease characterized by chronic non-alcoholic pancreatitis that presents with abdominal pain, steatorrhea, obstructive jaundice and responds well to steroid therapy and is seen in two subforms: type 1, which affects elderly males, involves other organs and has increased immunoglobin G4 (IgG4) levels and type 2, which affects both sexes equally but presents at a younger age and has no other organ involvement or increased IgG4 levels.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-103919/","orphanet_url":"https://www.orpha.net/en/disease/detail/103919","names":{"fr":{"name":"Pancréatite auto-immune","synonyms":["AIP"],"definition":"La pancréatite auto-immune est une maladie pancréatique rare caractérisée par une pancréatite chronique non-alcoolique se manifestant par une douleur abdominale, une stéatorrhée, un ictère obstructif. Cette maladie répond bien au traitement stéroïdien et est inclut 2 sous-formes : la pancréatite auto-immune de type 1 qui affect les hommes âgés, avec une atteinte extra-pancréatique et un taux élevé d'immunoglobine G4 (IgG4) et la pancréatite auto-immune de type 2 qui affecte les 2 sexes de manière égale, mais se manifeste à un âge plus jeune, sans atteinte extra-pancréatique ni élévation du taux IgG4 accrus."},"de":{"name":"Pankreatitis, autoimmune","synonyms":["Autoimmun-Pankreatitis"],"definition":"Die Autoimmun-Pankreatitis (AIP), eine seltene Pankreaserkrankung, ist gekennzeichnet durch chronische, nicht-alkoholische Pankreatitis, die sich mit Leibschmerzen, Steatorrhoe und Stauungsikterus äußert. Sie spricht gut auf Therapie mit Steroiden an und wird in zwei Unterformen aufgeteilt: (i) Typ 1 AIP (s. dort) tritt bei älteren Männern auf, bezieht auch andere Organe ein und zeigt erhöhte Immunglobulin G4 (IgG4)-Spiegel; (ii) Typ 2-AIP (s. dort) tritt in beiden Geschlechtern gleich auf und setzt in früherem Alter ein. Weitere Organe sind nicht betroffen, der IgG4-Spiegel ist nicht erhöht."},"es":{"name":"Pancreatitis autoinmune","synonyms":["AIP"],"definition":"Es una enfermedad pancreática poco frecuente caracterizada por una pancreatitis no alcohólica crónica que se presenta con dolor abdominal, esteatorrea, ictericia obstructiva y que responde bien a la terapia de esteroides; presenta dos subformas: el tipo 1, que afecta a varones de edad avanzada, implica a otros órganos y que presenta niveles incrementados de inmunoglobulina G4 (IgG4); y el tipo 2, que afecta a ambos sexos por igual pero se presenta a una edad más temprana sin afectación de otros órganos ni incremento de los niveles de IgG4."},"it":{"name":"Pancreatite autoimmune","synonyms":["AIP"],"definition":"La pancreatite autoimmune (AIP) è una malattia rara del pancreas, caratterizzata da pancreatite non alcolica cronica, che si manifesta con dolore addominale, steatorrea ed ittero ostruttivo. La malattia risponde bene alla terapia steroidea. Si può presentare in due forme: AIP tipo 1 (si veda questo termine), che colpisce le persone anziane di sesso maschile, coinvolge altri organi e si associa ad un aumento dei livelli dell'immunoglobulina G4 (IgG4); AIP tipo 2 (si veda questo termine), che interessa entrambi i sessi in eguale misura, esordisce a un'età più giovane, non coinvolge altri organi e non si associa ad un aumento dei livelli di IgG4."},"nl":{"name":"Auto-immune pancreatitis","synonyms":["AIP","Auto-immune alvleesklierontsteking"],"definition":"Een zeldzame aandoening van pancreas die gekarakteriseerd wordt door chronische, niet-alcoholische pancreatitis, zich presenteert met abdominale pijn, steatorroe en obstructieve geelzucht, goed reageert op steroïdtherapie, en waargenomen wordt in twee subtypes: type 1 dat oudere mannen treft, waarbij andere organen zijn betrokken en waarbij verhoogde immunoglobuline G4 (IgG4)-gehaltes voorkomen, en type 2 dat beide geslachten evenveel treft, aanvangt op k=jongere leeftijd, en geen betrokkenheid van andere organen of verhoogde IgG4-gehaltes vertoont."},"pt":{"name":"Pancreatite autoimune"},"pl":{"name":"Autoimmunologiczne zapalenie trzustki","synonyms":["AIP"]},"cs":{"name":"Autoimunitní pankreatitida","synonyms":["AIP"]},"uk":{"name":"Аутоімунний панкреатит","synonyms":["AIP"],"definition":"A rare pancreatic disease characterized by chronic non-alcoholic pancreatitis that presents with abdominal pain, steatorrhea, obstructive jaundice and responds well to steroid therapy and is seen in two subforms: type 1, which affects elderly males, involves other organs and has increased immunoglobin G4 (IgG4) levels and type 2, which affects both sexes equally but presents at a younger age and has no other organ involvement or increased IgG4 levels."},"zh":{"name":"自身免疫性胰腺炎","synonyms":["AIP"],"definition":"自身免疫性胰腺炎是一种罕见的胰腺疾病，以腹痛、脂肪痢,阻塞性黄疸和类固醇治疗反应良好的慢性非酒精性胰腺炎为特征,出现在两个子类型:1型影响老年男性,包括其他器官受累和增加了IgG4水平增加，2型患者无性别差别但更年轻,也没有其他器官参与或增加IgG4水平。"},"tr":{"name":"Otoimmün pankreatit","synonyms":["AIP"]}},"xrefs":[{"source":"ICD-11","code":"DC33","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"2057951941"},{"source":"MONDO","code":"0015175","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"15175","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D000081012","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2609129","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10911","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10069002","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97935,"name":"Rare gastroenterologic disease"},"parents":[{"orpha":101937,"name":"Rare pancreatic disease","classifications":[152]}],"children":[{"orpha":280302,"name":"Autoimmune pancreatitis type 1","type":"Clinical subtype"},{"orpha":280315,"name":"Autoimmune pancreatitis type 2","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}