{"orpha":280302,"name":"Autoimmune pancreatitis type 1","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["AIP type 1","IgG4-related pancreatitis","Lymphoplasmacytic sclerosing pancreatitis"],"definition":"Type 1 autoimmune pancreatitis is a form of autoimmune pancreatitis seen in elderly males (>60 years) and presenting with abdominal pain, steatorrhea, obstructive jaundice and other organ (bile duct, kidneys and retroperitoneum) involvement. It is thought to be due to an immunoglobulin G4 (IgG4)-associated systemic disease.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-280302/","orphanet_url":"https://www.orpha.net/en/disease/detail/280302","names":{"fr":{"name":"Pancréatite auto-immune type 1","synonyms":["AIP type 1","Pancréatite associée à IgG4","Pancréatite sclérosante lymphoplasmocytaire"],"definition":"La pancréatite auto-immune de type 1 est une forme de pancréatite auto-immune rapportée chez des hommes âgés (>60 ans) et se manifestant par une douleur abdominale, une stéarrohée, un ictère obstructif avec une atteinte extra-pancréatique (la voie biliaire, les reins et le rétropéritoine). On pense qu'elle serait une maladie systémique associée aux IgG4."},"de":{"name":"Pankreatitis, autoimmune, Typ 1","synonyms":["AIP Typ 1","Autoimmun-Pankreatitis Typ 1","Pankreatitis, IgG4-assoziierte","Pankreatitis, lymphoplasmozytäre sklerosierende"],"definition":"Der Typ 1 der Autoimmun-Pankreatitis, eine Unterform der Autoimmun-Pankreatitis (s. dort), wird bei älteren Männern (>60 Jahre) gesehen und manifestiert sich mit Leibschmerzen, Steatorrhoe, obstruktivem Ikterus und Beteiligung anderer Organe (Gallengänge, Nieren, Retroperitoneum). Vermutlich handelt es sich um eine systemische Immunglobulin G4 (IgG4)-assoziierte Krankheit."},"es":{"name":"Pancreatitis autoinmune tipo 1","synonyms":["AIP tipo 1","Pancreatitis asociada a IgG4","Pancreatitis linfoplasmocitaria esclerosante"],"definition":"La pancreatitis autoinmune tipo 1 es una forma de pancreatitis autoinmune observada en varones de avanzada edad (>60 años) y que se presenta con dolor abdominal, esteatorrea, ictericia obstructiva y afectando a otros órganos (conducto biliar, riñones y retroperitoneo). Se cree que está causada por una enfermedad sistémica asociada a la inmunoglobulina G4 (IgG4)."},"it":{"name":"Pancreatite autoimmune, tipo 1","synonyms":["AIP, tipo 1","Pancreatite IgG4-correlata","Pancreatite sclerosante linfoplasmocitaria"],"definition":"La pancreatite autoimmune tipo 1 è una forma di pancreatite autoimmune che si osserva nei maschi anziani (> 60 anni) e si manifesta con dolore addominale, steatorrea, ittero ostruttivo e coinvolge altri organi (dotto biliare, reni, retroperitoneo). Si ritiene che sia dovuta a una malattia sistemica correlata alla IgG4 (immunoglobulina 4)."},"nl":{"name":"Auto-immune pancreatitis type 1","synonyms":["AIP type 1","IgG4-gerelateerde pancreatitis","Lymfoplasmacytaire scleroserende pancreatitis"],"definition":"Een vorm van auto-immune pancreatitis, waargenomen bij oudere mannen (ouder dan 60 jaar) en gekenmerkt door abdominale pijn, steatorroe, obstructieve geelzucht, en betrokkenheid van andere organen (galwegen, nieren en retroperitoneum). Het is vermoedelijk te wijten aan een immunoglobuline G4 (IgG4)-geassocieerde systemische ziekte."},"pt":{"name":"Pancreatite autoimune 1","synonyms":["AIP tipo 1","Pancreatite esclerosante linfoplasmocítica"]},"pl":{"name":"Autoimmunologiczne zapalenie trzustki typu 1","synonyms":["AIP typu 1","Limfoplazmatyczne stwardniające zapalenie trzustki"]},"cs":{"name":"Autoimunitní pankreatitida, typ 1","synonyms":["AIP typ 1","IgG4 asociovaná pankreatitida","Lymfoplazmocytární sklerozující pankreatitida"]},"uk":{"name":"Аутоімунний панкреатит, тип 1","synonyms":["AIP тип 1","Панкреатит, повязаний з IgG4","Лімфоплазмоцитарний склерозуючий панкреатит"],"definition":"Type 1 autoimmune pancreatitis is a form of autoimmune pancreatitis seen in elderly males (>60 years) and presenting with abdominal pain, steatorrhea, obstructive jaundice and other organ (bile duct, kidneys and retroperitoneum) involvement. It is thought to be due to an immunoglobulin G4 (IgG4)-associated systemic disease."},"zh":{"name":"自身免疫性胰腺炎1型","synonyms":["淋巴浆硬化性胰腺炎"],"definition":"自身免疫性胰腺炎1型是多见于老年男性（ inf 60岁）的自身免疫性胰腺炎，表现为腹痛、脂肪泻、梗阻性黄疸，可累及其他器官（胆管、肾脏和腹膜后），是与免疫球蛋白G4（IgG4）相关的全身性疾病。"},"tr":{"name":"Otoimmün pankreatit tip 1","synonyms":["AIP tip 1","IgG4-ilişkili pankreatit","Lenfoplazmasitik sklerozan pankreatit"]}},"xrefs":[{"source":"ICD-10","code":"K86.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DC33","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"614401663"},{"source":"MONDO","code":"0017227","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4302243","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97935,"name":"Rare gastroenterologic disease"},"parents":[{"orpha":103919,"name":"Autoimmune pancreatitis","classifications":[152]},{"orpha":596448,"name":"IgG4-related systemic disease","classifications":[196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}