{"orpha":447774,"name":"Secondary sclerosing cholangitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, biliary tract disease characterized by development of sclerosing cholangitis due to a known primary insult to the biliary tree, including infections, autoimmune disease, exposure to toxic agents, obstructive and ischemic injuries. Patients may be initially asymptomatic with only elevated alkaline phosphatase and gamma glutamyltransferase levels. Later presentation includes abdominal pain, jaundice, pruritus, fever and bacterial cholangitis from ascending infection.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-447774/","orphanet_url":"https://www.orpha.net/en/disease/detail/447774","names":{"fr":{"name":"Cholangite sclérosante secondaire","definition":"Maladie rare des voies biliaires caractérisée par le développement d'une cholangite sclérosante due à une atteinte inflammatoire primaire connue des voies biliaires, telle que des infections, une maladie auto-immune, une exposition à des agents toxiques, des lésions obstructives et ischémiques. Au début de la maladie, les patients peuvent être asymptomatiques et présenter seulement des taux élevés de phosphatases alcalines et de gamma-glutamyl transférase. Plus tard, les symptômes qu'ils présentent incluent des douleurs abdominales, un ictère, un prurit, une fièvre et une cholangite ascendante due à une infection bactérienne."},"de":{"name":"Sklerosierende Cholangitis, sekundäre","definition":"Eine seltene Erkrankung der Gallenwege, die durch die Entwicklung einer sklerosierenden Cholangitis infolge einer bekannten primären Schädigung des biliären Systems gekennzeichnet ist, einschließlich Infektionen, Autoimmunerkrankungen, Exposition gegenüber toxischen Substanzen, obstruktiven und ischämischen Verletzungen. Die Patienten können zunächst asymptomatisch sein und lediglich erhöhte Werte der alkalischen Phosphatase und der Gamma-Glutamyltransferase aufweisen. Später treten Bauchschmerzen, Gelbsucht, Juckreiz, Fieber und bakterielle Cholangitis aufgrund einer aufsteigenden Infektion auf."},"es":{"name":"Colangitis esclerosante secundaria","definition":"Es una enfermedad poco frecuente de las vías biliares caracterizada por el desarrollo de colangitis esclerosante debido a una agresión primaria y conocida del árbol biliar, tal como infecciones, enfermedades autoinmunes, exposición a agentes tóxicos, lesiones obstructivas e isquémicas. Los pacientes pueden mantenerse inicialmente asintomáticos aunque presentan niveles elevados de fosfatasa alcalina y gamma-glutamiltransferasa. La presentación tardía incluye dolor abdominal, ictericia, prurito, fiebre y colangitis bacteriana por infección ascendente."},"it":{"name":"Colangite sclerosante secondaria","definition":"È una malattia rara delle vie biliari, caratterizzata da colangite sclerosante secondari ad un danno primitivo dell'albero biliare (infezioni, malattie autoimmuni, esposizione ad agenti tossici, e danni ostruttivi ed ischemici). Inizialmente i pazienti possono essere asintomatici e presentare solo un aumento della fosfatasi alcalina e della gamma glutamil-transferasi. Successivamente insorgono dolore addominale, ittero, prurito, febbre e colangite batterica secondaria a un'infezione ascendente."},"nl":{"name":"Secundaire scleroserende cholangitis","definition":"Een zeldzame ziekte van de galwegen, gekarakteriseerd door ontwikkeling van scleroserende cholangitis als gevolg van een gekende primair letsel aan het galwegenstelsel, waaronder infecties, auto-immuunziekte, blootstelling aan toxische agentia, obstructieve en ischemische verwondingen. Patiënten kunnen initieel asymptomatisch zijn, met enkel verhoogde gehaltes van alkalische fosfatase en gamma-glutamyltransferase. Latere presentatie omvat buikpijn, geelzucht, pruritus, koorts en bacteriële cholangitis door een opstijgende infectie."},"pt":{"name":"Colangite esclerosante secundária"},"pl":{"name":"Wtórne twardniające zapalenie dróg żółciowych"},"cs":{"name":"Sekundární sklerotizující cholangitida"},"uk":{"name":"Вторинний склерозуючий холангіт","definition":"A rare, biliary tract disease characterized by development of sclerosing cholangitis due to a known primary insult to the biliary tree, including infections, autoimmune disease, exposure to toxic agents, obstructive and ischemic injuries. Patients may be initially asymptomatic with only elevated alkaline phosphatase and gamma glutamyltransferase levels. Later presentation includes abdominal pain, jaundice, pruritus, fever and bacterial cholangitis from ascending infection."},"zh":{"name":"继发型硬化性胆管炎","definition":"一种罕见的胆道疾病，其特征在于由于原发性的胆道树损害导致的进展性硬化性胆管炎，损害包括感染、自身免疫性疾病、接触有毒物质、阻塞性和缺血性损伤。患者可能最初无症状，仅碱性磷酸酶和γ-谷氨酰转移酶水平升高。后续的表型包括由上行感染引起的腹部感染，黄疸，瘙痒，发烧和细菌感染。"},"tr":{"name":"Sekonder sklerozan kolanjit"}},"xrefs":[{"source":"ICD-10","code":"K83.0","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"DC13","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1876249967"},{"source":"MONDO","code":"0018647","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0400978","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":57146,"name":"Rare hepatic disease"},"parents":[{"orpha":447771,"name":"Sclerosing cholangitis","classifications":[183,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}