Primary Sclerosing Cholangitis: Diagnostic Criteria

Cazzagon, Nora; Sarcognato, Samantha; Catanzaro, Elisa; Bonaiuto, Emanuela; Peviani, Matteo; Pezzato, Francesco; Motta, Raffaella

doi:10.3390/tomography10010005

Cited by 2 publications

(1 citation statement)

References 79 publications

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“…Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease characterized by multifocal fibrosis of intra- and/or extrahepatic bile ducts, which eventually causes narrowing and even blockage, leading to the formation of multifocal strictures alternating with dilatations [ 1 , 2 ]. PSC is very rare, affecting 0-16 people per 100,000, and ultimately leads to cirrhosis and end-stage liver disease [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

Primary Sclerosing Cholangitis Associated With Ulcerative Colitis Coexisting With Cholangiocarcinoma: A Case Report

Hamid,

Alshoabi,

Binnuhaid

et al. 2024

Cureus

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Primary sclerosing cholangitis (PSC) is a rare chronic inflammatory disease in which multifocal fibrosis of bile ducts causes eventually narrowing and even blocking, forming multifocal strictures alternated to dilatations. Here, we reported an extremely rare case of PSC associated with ulcerative colitis (UC) and coexisting with cholangiocarcinoma in a 33-year-old male presented with right upper quadrant pain and dark urine. Liver function tests were deranged, and ERCP found a beaded cholangiography appearance due to multifocal bile duct strictures alternating with normal and dilated segments of the common hepatic duct and the intrahepatic bile ducts. We aim to document this typical case of PSC associated with UC and coexisted with cholangiocarcinoma to add the existing data on these rare pathologies.

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Section: Introductionmentioning

confidence: 99%

Primary Sclerosing Cholangitis Associated With Ulcerative Colitis Coexisting With Cholangiocarcinoma: A Case Report

Hamid,

Alshoabi,

Binnuhaid

et al. 2024

Cureus

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Quantitative Magnetic Resonance Cholangiopancreatography Scoring and Its Predictive Value for Outcomes in Adults with Primary Sclerosing Cholangitis

Mandea,

Iacob,

Grasu

et al. 2024

JCM

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Background: Primary sclerosing cholangitis (PSC) is an immune-mediated disease that has an unfavorable prognosis and needs a liver transplant (LT). The aim of this paper was to show the usefulness of the Majoie classification on magnetic resonance cholangiopancreatography (MRCP) images in assessing the prognosis in adult patients with PSC. Methods: Our work presents a retrospective monocentric study performed on 64 adult patients with PSC of the large bile ducts. Two radiologists evaluated the MRCP of diagnosis and calculated MRCP scores using the Majoie classification. Liver-related outcome (LT or liver-related death) was marked as a primary endpoint. Results: Univariate analysis showed that patients with more severe lesions (sum score of intrahepatic and extrahepatic ducts > 3) had a lower age at diagnosis, of 37.2 years, complicated with liver cirrhosis (53.1% of patients) and recurrent cholangitis (28.1%) p < 0.05, without significant differences in mortality, association with IBD or LT. Concordance analysis between MRCP prognostic scores and progression to a PSC-related event showed a moderate relationship (c-statistic 0.662), and a good AUROC was observed for the UKPSC score (0.893) and the MRS (0.936). Conclusions: In the study, we observed a good correlation between the imaging scores based on the Majoie classification and the evolution of the patients. These scores were outperformed by the UKPSC, MRS, and PREsTo clinical models. Their utility was best in predicting recurrent cholangitis.

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Primary Sclerosing Cholangitis: Diagnostic Criteria

Cited by 2 publications

References 79 publications

Primary Sclerosing Cholangitis Associated With Ulcerative Colitis Coexisting With Cholangiocarcinoma: A Case Report

Primary Sclerosing Cholangitis Associated With Ulcerative Colitis Coexisting With Cholangiocarcinoma: A Case Report

Quantitative Magnetic Resonance Cholangiopancreatography Scoring and Its Predictive Value for Outcomes in Adults with Primary Sclerosing Cholangitis

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