{"orpha":300552,"name":"Follicular cholangitis and pancreatitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Follicular pancreatocholangitis"],"definition":"Follicular cholangitis and pancreatitis is a rare pancreatobiliary disease characterized by marked duct-centered lymphoid follicular inflammation that develops in both biliary and pancreatic ductal systems, mainly affecting the hilar bile ducts and the pancreatic head. Patients present with jaundice, abdominal pain, liver dysfunction, pruritus and/or weight loss. Histology shows lymphoplasmacytic infiltration with formation of numerous, large lymphpoid follicles around the affected bile and pancreatic ducts.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-300552/","orphanet_url":"https://www.orpha.net/en/disease/detail/300552","names":{"fr":{"name":"Pancréatite et cholangite folliculaire","synonyms":["Pancréatocholangite folliculaire"],"definition":"Maladie pancréatobiliaire rare caractérisée par une inflammation folliculaire lymphoïde marquée, se développant autour des canaux biliaires et pancréatiques, affectant principalement les canaux situés dans le hile du foie et la tête du pancréas. Les symptômes observés chez les patients sont un ictère, des douleurs abdominales, un insuffisance hépatique, un prurit et/ou une perte de poids. L'histologie met en évidence une infiltration lymphoplasmocytaire avec formation de nombreux follicules lymphoïdes de grande taille autour des canaux biliaires et pancréatiques affectés."},"de":{"name":"Cholangitis und Pankreatitis, follikuläre","synonyms":["Pankreatocholangitis, follikuläre"]},"es":{"name":"Colangitis folicular y pancreatitis","synonyms":["Pancreatocolangitis folicular"],"definition":"Es una enfermedad pancreatobiliar poco frecuente caracterizada por una marcada inflamación folicular linfoide ductular, que se desarrolla en ambos sistemas ductales tanto biliar como pancreático, afectando principalmente a los conductos biliares hiliares y a la cabeza pancreática. Los afectados presentan ictericia, dolor abdominal, disfunción hepática, prurito y/o pérdida de peso. Histológicamente, se observa una infiltración linfoplasmocitaria con formación de numerosos folículos linfoides grandes alrededor de los conductos biliares y pancreáticos afectados."},"it":{"name":"Colangite follicare e pancreatite","synonyms":["Pancreatocolangite follicolare"],"definition":"La colangite follicolare associata a pancreatite è una malattia pancreatobiliare rara, caratterizzata da marcata infiammazione follicolare linfoide duttale, che si manifesta nel sistema duttale biliare e pancreatico, con interessamento prevalente dei dotti biliari ilari e della testa del pancreas. I pazienti presentano ittero, dolore addominale, disfunzione epatica, prurito e/o perdita di peso. L'esame istologico evidenzia infiltrazione linfoplasmacitaria con formazione di numerosi follicoli linfoidi di ampie dimensioni attorno ai dotti biliari e pancreatici interessati."},"nl":{"name":"Folliculaire cholangitis en pancreatitis","synonyms":["Folliculaire pancreatocholangitis"],"definition":"Folliculaire cholangitis en pancreatitis is een zeldzame pancreatobiliaire ziekte, en wordt gekarakteriseerd door duidelijke lymfoïde folliculaire inflammatie die zich ontwikkelt in zowel gal- als pancreaskanalen en die voornamelijk de hilaire galwegen en de pancreaskop treft. Patiënten vertonen geelzucht, buikpijn, leverdisfunctie, pruritus en/of gewichtsverlies. Histologie toont lymfoplasmacytaire infiltratie met vorming van talloze, grote lymfoïde follikels rond de getroffen gal- en pancreaswegen."},"pt":{"name":"Colangite folicular e pancreatite"},"pl":{"name":"Pęcherzykowe zapalenie przewodów żółciowych i trzustki","synonyms":["Follicular pancreatocholangitis"],"definition":"*Pęcherzykowe zapalenie przewodów żółciowych i trzustki jest rzadką chorobą trzustki i dróg żółciowych, która charakteryzuje się wyraźnym limfoidalnym pęcherzykowym stanem zapalnym, rozwijającym się wokół przewodów żółciowych i trzustkowych i dotyczącym głównie przewodów zlokalizowanych we wnęce wątroby i głowie trzustki. Objawy obserwowane u pacjentów to: żółtaczka, ból brzucha, zaburzenia czynności wątroby, świąd i/lub utrata masy ciała. W badaniu histologicznym obserwuje się naciek limfoplazmocytowy z tworzeniem licznych, dużych pęcherzyków limfoidalnych wokół dotkniętych chorobą przewodów żółciowych i trzustkowych."},"cs":{"name":"Folikulární cholangitida a pankreatitida","synonyms":["Folikulární pankreatocholangitida"]},"uk":{"name":"Фолікулярний холангіт і панкреатит","synonyms":["Фолікулярний панкреатохолангіт"],"definition":"Follicular cholangitis and pancreatitis is a rare pancreatobiliary disease characterized by marked duct-centered lymphoid follicular inflammation that develops in both biliary and pancreatic ductal systems, mainly affecting the hilar bile ducts and the pancreatic head. Patients present with jaundice, abdominal pain, liver dysfunction, pruritus and/or weight loss. Histology shows lymphoplasmacytic infiltration with formation of numerous, large lymphpoid follicles around the affected bile and pancreatic ducts."},"zh":{"name":"滤泡性胆管炎和胰腺炎","synonyms":["滤泡性胰腺胆管炎"],"definition":"滤泡性胆管炎和胰腺炎是一种罕见的胰胆管疾病，其特征是在胆管和胰管系统均发生明显的以导管为中心的淋巴滤泡性炎症，主要累及肝门胆管和胰头。患者表现为黄疸、腹痛、肝功能不全、瘙痒和/或体重减轻。组织学显示在受累胆管和胰管周围淋巴浆细胞浸润，形成大量大淋巴滤泡。"},"tr":{"name":"Foliküler kolanjit ve pankreatit","synonyms":["Foliküler pankreatokolanjit"]}},"xrefs":[{"source":"ICD-10","code":"K83.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"K86.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DC33","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0017589","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4749906","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97935,"name":"Rare gastroenterologic disease"},"parents":[{"orpha":101941,"name":"Rare biliary tract disease","classifications":[183]},{"orpha":101937,"name":"Rare pancreatic disease","classifications":[152]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":5.0,"area":"Worldwide","status":"Validated","source":"22211284[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}