An Overview on Primary Sclerosing Cholangitis

Abstract

Primary sclerosing cholangitis is a progressive liver disease characterized by chronic inflammation leading to liver fibrosis and cirrhosis. Even though the exact pathogenesis is still unclear, a combination of autoimmune, environmental, and ischemic factors could explain certain aspects of the disease. The most important diagnostic step is cholangiography, which can be obtained either by endoscopic retrograde cholangiopancreatography (ERCP), magnetic resonance cholangiography (MRCP as the gold standard), or percutaneous transhepatic cholangiography. It shows multifocal short biliary duct strictures leading to the “beaded” aspect. Cholangiocarcinoma and colorectal adenocarcinoma are the most feared complications in patients with Primary sclerosing cholangitis (PSC). Continuous screening consists of annual clinical, biochemical, and ultrasound assessments in asymptomatic patients and annual colonoscopy in patients with PSC and inflammatory bowel disease. In newly diagnosed patients with PSC, colonoscopy is mandatory and, if negative, then, a repeat colonoscopy should be performed in 3–5 years. The lack of efficient curative medical treatment makes invasive treatments such as liver transplant and endoscopy the mainstream for managing PSC and its complications. Until now, even though only ursodeoxycholic acid has shown a moderate clinical, biochemical, and even histological improvement, it has no significant influence on the risk of cholangiocarcinoma, liver transplant need, or death risk and it is no longer recommended in treating early PSC. Further studies are in progress to establish the effect of molecular-targeted therapies in PSC.

Selected transgenic murine models of human autoimmune liver diseases

“…One of the mechanisms leading to autoimmune disease development starts with leakage through the colon membrane, which in turn causes lipopolysaccharides (LPS) to enter the liver and stimulate Toll-like receptors. As a result of these events, the liver immune response is induced, resulting in leukocyte activation and infiltration into the liver parenchyma [19]. Importantly, Tang and coworkers reported substantial differences in the composition and function of the gut microbiome between treatment-naive PBC subjects and healthy controls.…”

Choledochoscopy: An update

“…In bacterial cholangitis superimposed, temporary drainage and flushing measures to keep the biliary ducts patent can be performed. This includes the use of naso-biliary tubes for drainage, biliary lavage for decanting and flushing[ 68 ], as well as percutaneous transhepatic cholangioplasty for relief of jaundice[ 69 ].…”

Cholestatic liver disease: Practice guidelines from the Saudi Association for the Study of Liver diseases and Transplantation

“…Patients with PSC, who developed bacterial cholangitis related to dominant stricture, respond mostly to therapeutic drainage of the obstruction, along with antibiotics. Long-term prophylactic antibiotics are often considered in recurrent bacterial cholangitis, which can be severe, and considered as a primary indication for LT.[ 89 ] Due to the lack of effective nonsurgical treatment for PSC and associated complications, more invasive therapy such as endoscopy and liver transplants are commonly considered on a case-by-case basis.…”

“…Before attempting endoscopic therapy, fluorescence in-situ hybridization (FISH), endoscopic biopsy, and/or brush cytology must be performed to exclude the possibility of malignancy. [ 89 ]…”