Biliary endoscopy in the management of primary sclerosing cholangitis and its complications

Abstract

Primary sclerosing cholangitis (PSC) is a chronic, idiopathic, cholestatic liver disease characterized by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts. It can affect individuals of all age groups and gender, has no established pharmacotherapy, and is associated with a variety of neoplastic ( e.g. cholangiocarcinoma) and non-neoplastic ( e.g. dominant strictures) hepatobiliary complications. Given these considerations, endoscopy plays a major role in the care of patients with PSC. In this review, we discuss and provide updates regarding endoscopic considerations in the management of hepatobiliary manifestations and complications of PSC. Where evidence is limited, we suggest pragmatic approaches based on currently available data and expert opinion.

A Rare Case of 18-year-old female with Primary Sclerosing Cholangitis, Mixed Connective Tissue Disease, Short Stature

“…Even though the mean duration of the stent patency is about 12 months and needs close and regular monitoring and prolonged corticosteroid therapy may give serious side effects, they are still considered an effective treatment modality for this patient that does not have resources for liver transplantation. (12) Her quality of life was improved but the patient still had no menstruation and her secondary sexual sign did not develop. Fibrotic autoimmune diseases are characterized by an inflammatory process, which induces and sustains robust fibrosis.…”

Biliary Tract Malignancies Related to Gallbladder Polyps, Primary Sclerosing Cholangitis, and Choledochal Cysts

“…The caveat is increased risk for tumor seeding and subsequent peritoneal metastases leading to exclusion from liver transplant (LT) candidacy as per Mayo Clinic protocol. 16 Currently, there is a lack of consensus on screening of patients with PSC for CCA. The American Association for the Study of Liver Diseases states that "in the absence of evidence based information, many clinicians screen patients with an imaging study plus a CA 19-9 at annual intervals."…”

“…The addition of fine needle aspiration increases the sensitivity and specificity to 84% and 100%, respectively. The caveat is increased risk for tumor seeding and subsequent peritoneal metastases leading to exclusion from liver transplant (LT) candidacy as per Mayo Clinic protocol 16 . Currently, there is a lack of consensus on screening of patients with PSC for CCA.…”

Endoscopic ultrasound in chronic liver disease

“…With advances in MRI technology, magnetic resonance cholangiopancreatography (MRCP) has generally replaced endoscopic retrograde cholangiopancreatography (ERCP) as the initial diagnostic as well as surveillance modality for primary sclerosing cholangitis (PSC)[ 49 , 50 ]. However, the sensitivity of MRCP is not without limitation, with one systematic review suggesting a sensitivity of MRCP of only 86%[ 51 ].…”