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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease sclerosing cholangitis
Comorbidity C0566602|primary sclerosing cholangitis
Sentences 32
PubMedID- 24765316 Primary sclerosing cholangitis is a progressive disease, which is rare in pregnancy.
PubMedID- 22988518 Primary sclerosing cholangitis (psc) is a chronic cholestatic disease of intrahepatic and extrahepatic biliary ducts, characterized by chronic periductal inflammation and sclerosing of the ducts, which results in segmental stenosis of bile ducts, cholestasis, and fibrosis [1].
PubMedID- 25646372 We analysed patients with igg4-rd, patients with primary sclerosing cholangitis (psc) and elevated igg4 (a subset of patients with psc who have an elevated serum igg4 level but no histological or radiological evidence of igg4-rd), and healthy controls in a uk cohort.
PubMedID- 20531971 Primary sclerosing cholangitis (psc) is a rare cholestatic liver disease with the histological features of chronic inflammation and fibrotic obliteration of the hepatic biliary tree, resulting in bile stasis and hepatic fibrosis.1 it commonly co-exists with autoimmune hepatitis (aih) as a part of hepatic overlap syndrome.
PubMedID- 24634588 Primary sclerosing cholangitis (psc) is a chronic fibroinflammatory syndrome linking chronic inflammation to carcinogenesis.33 there is an increased frequency of gallbladder mass lesions in patients with psc; the basis presumably relates to the ongoing inflammation, facilitating a metaplasia–dysplasia–carcinoma sequence.34 the strong correlation between gallbladder dysplasia/adenocarcinoma and bile duct dysplasia/cholangiocarcinoma supports the concept of a neoplastic “field effect” along the intrahepatic and extrahepatic biliary tract in psc patients.34 those with psc therefore should undergo annual gallbladder cancer surveillance, via abdominal ultrasound screening, for masses.35,36 cholecystectomy should be performed for lesions >0.8 cm.
PubMedID- 22927860 Our objective was to describe a 14-year-old patient with bbs who presented with crohn disease (cd), primary sclerosing cholangitis (psc), and thyroiditis in the context of a cohort review at sainte-justine hospital and to alert clinicians to the increased risk of autoimmune disorders in these patients.
PubMedID- 24750423 Primary sclerosing cholangitis (psc) is a chronic progressive disease of unknown aetiology characterized by fibrosclerotic destruction of the bile ducts resulting in multifocal stricturing of the biliary tree (mendes & lindor 2010; hirschfield et al.
PubMedID- 21356211 Pv hepatocytes, isolated from benign liver resections, normal donor tissue and biliary cirrhosis (primary biliary cirrhosis and primary sclerosing cholangitis) showed significant increase in ros accumulation during hypoxia (fig.
PubMedID- 26357612 Autoimmune hepatitis (aih), primary biliary cirrhosis (pbc) and primary sclerosing cholangitis (psc) are three major types of autoimmune diseases localized in the liver or autoimmune liver diseases, whereas granulomatous diseases such as sarcoidosis and connective tissue diseases such as systemic lupus erythematosus, rheumatoid arthritis and primary sjögren syndrome (pss) are the major systemic autoimmune diseases with liver involvement.
PubMedID- 21286351 It needs to be differentiated from primary sclerosing cholangitis (psc) and cholangiocarcinoma, as it results in completely different therapeutic strategy.
PubMedID- 22693517 Primary sclerosing cholangitis (psc) was the single most common diagnosis in the non-igg4-rd group.
PubMedID- 26431155 Rna pools of 15 patients with primary sclerosing cholangitis (psc) and eight patients with cholangiocarcinoma (cc) identified several deregulated mirnas in bile.
PubMedID- 23319033 Primary sclerosing cholangitis, sjögren's syndrome, psoriasis, and vitiligo were encountered in one patient (0.2%) each.
PubMedID- 20975735 Primary sclerosing cholangitis 44. this abnormal expression of pdc-e2 may depend on self-antigens being presented by cholangiocytes after binding to hla molecules, although hla class ii on cholangiocytes have more an intrahepatic basolateral than luminal surface expression 56–60 and anyway, are expressed only weakly and in the early stages of disease 60. this observation could be also secondary to immune complexes deposition rather than membrane protein expression.
PubMedID- 21059205 Myasthenia gravis may occur together with primary sclerosing cholangitis in the same patient.
PubMedID- 22886419 Chronic cholelithiasis, primary sclerosing cholangitis (psc), primary biliary cirrhosis (pbc), and biliary atresia are examples of cholestatic human liver diseases, as reviewed elsewhere.1, 2 cholestasis results in bile duct injury caused by the accumulation of toxic hydrophobic bile acids, which also damages the periportal hepatocytes.3 in response to injury, both biliary epithelial cells (becs) and hepatocytes will proliferate to compensate for their respective loss.
PubMedID- PMC4070603 Concomitant primary sclerosing cholangitis (psc) and disease extent are the only factors that influence the start and periodicity of colonoscopy surveillance in these patients.
PubMedID- 21103219 Diagnosis of primary sclerosing cholangitis is established by the detection of characteristic alterations in the biliary tree as seen with ercp or percutaneous transhepatic cholangiography or by the detection of periductal fibrosis on liver biopsy.
PubMedID- 26425607 One of the most dreaded complications of primary sclerosing cholangitis is cholangiocarcinoma, and this must always be considered in patients such as that presented here.
PubMedID- 21209818 The diagnosis of primary sclerosing cholangitis (psc) in this patient is established by the biochemical profile of chronic cholestasis, typical strictures and pruning of the biliary tree upon cholangiography, and ring fibrosis around the bile ducts in liver biopsy.
PubMedID- 24073337 One of the best examples offered was represented by primary sclerosing cholangitis occurring in uc: in a subset of patients where the presence (in sera and colonic mucosa) of anticolonic mucosa autoantibodies that cross-react with biliary epithelium has been identified [21].
PubMedID- 24495412 Primary sclerosing cholangitis (psc) can cause benign biliary stricturing and is the most common risk factor for cca in western populations, with rates of 8-30% reported in clinical follow-up or liver explant specimens after transplant.
PubMedID- 20740204 Diseases such as myasthenia gravis, lupus erythematosis, rheumatoid arthritis, thyroiditis and sjögren's syndrome, dermatomyositis, primary sclerosing cholangitis, multiple sclerosis and pernicious anaemia can be seen in combination with thymomas [2].
PubMedID- 20811628 Primary sclerosing cholangitis (psc) is a chronic inflammatory condition of the intra- and extrahepatic bile ducts with a prevalence of approximately 10 per 100,000 in western countries [10], [11].
PubMedID- 21994888 Primary biliary cirrhosis (pbc), primary sclerosing cholangitis (psc), and hepatolithiasis in adults and biliary atresia and choledochal cyst in infants are biliary diseases in which different anatomical levels of the biliary tree are specifically affected and characterized by cholangiopathy.
PubMedID- 22087157 Liver disorders such as autoimmune hepatitis, elevation of liver enzyme levels, primary biliary cirrhosis, nonspecific hepatitis, primary sclerosing cholangitis, and nonalcoholic fatty liver disease have been reported in patients with cd.
PubMedID- 24754367 Autoimmune hepatitis and primary sclerosing cholangitis in children and adolescents.
PubMedID- 26557943 There are several established risk factors for cc, such as primary sclerosing cholangitis (psc), which is the commonest known predisposing factor for cc in the western world [5].
PubMedID- 21767410 Primary sclerosing cholangitis (psc) is a chronic cholestatic liver disease of unknown etiology [1] characterized by classic findings of multiple segmental strictures in the intra- and extra-hepatic bile ducts secondary to inflammation and obliterative fibrosis.
PubMedID- 22701310 A family history of crc, the presence of primary sclerosing cholangitis, backwash ileitis, and, in some studies, young age at onset of colitis increase further the risk of ibd-associated crc [5–8].
PubMedID- 24552185 Few cases have reported an association of polymyositis with cd, although the occurrence of polymyositis, alopecia universalis, and primary sclerosing cholangitis was observed in a male cd patient in germany [8], and hall documented a case of focal myositis involving the left gastrocnemius muscle [9].
PubMedID- 25788760 One patient was staged as t0 and was transplanted for primary sclerosing cholangitis and ihc was identified in the explanted liver.

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