Primary Sclerosing Cholangitis. A narrative review

Primary sclerosing cholangitis (PSC) is a rare, chronic liver pathology characterized by inflammation and fibrosis of the bile ducts, whose evolution can lead to cirrhosis, portal hypertension and end-stage liver disease. Its etiology is unknown, but it has been related to genetic and autoi...

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Bibliographic Details
Main Authors: Villa Gómez, Cristian Camilo, Velilla Aguirre, Daniel Stiven, Lopera Restrepo, Laura Camila, Hoyos Duque, Sergio Iván
Format: Article
Language:Spanish
Published: Universidad de Antioquia 2020-07-01
Series:Iatreia
Subjects:
Online Access:https://revistas.udea.edu.co/index.php/iatreia/article/view/339209/20802972
Description
Summary:Primary sclerosing cholangitis (PSC) is a rare, chronic liver pathology characterized by inflammation and fibrosis of the bile ducts, whose evolution can lead to cirrhosis, portal hypertension and end-stage liver disease. Its etiology is unknown, but it has been related to genetic and autoinflammatory factors. In addition, it has a very close relationship with inflammatory bowel disease. Its clinical presentation is nonspecific, the main symptoms are pruritus and fatigue. The standard test for diagnosis is magnetic resonance cholangiopancreatography (MRCP), where an annular aspect is observed caused by short multifocal stenoses with alternating normal or dilated segments. Currently, there is no pharmacological treatment that can prolong the survival without liver transplantation in PSC. Symptomatic treatment is warranted, especially for pruritus. The only curative treatment that is currently available is liver transplantation, although there is a risk of recurrence of the disease. The monitoring of intestinal inflammatory disorders (IID), malignancy andmetabolic bone disease in these patients is very important. It has been seen that some factors, such as early diagnosis, are good prognosis for the disease.
ISSN:0121-0793
2011-7965