Current diagnosis and management of primary sclerosing cholangitis

Abstract

Primary sclerosing cholangitis (PSC) is an important liver disease with major morbidity and mortality. The diagnosis of PSC is confirmed by magnetic resonance cholangiopancreaticography, and endoscopic retrograde cholangiopancreaticography is performed in patients needing therapeutic endoscopy. As a result of the unknown cause of the disease, current medical therapies are unsatisfactory. Nevertheless, high-dose ursodeoxycholic acid should be recommended for treatment of PSC patients because there is a trend toward increased survival. Dominant bile duct stenoses should be treated endoscopically. However, liver transplantation continues to be the only therapeutic option for patients with advanced disease. Estimation of prognosis and timing of liver transplantation should be determined individually for each PSC patient on the basis of all results. The diagnosis and treatment of cholangiocarcinoma (CC) still remain a challenge in PSC patients. Early diagnosis of CC certainly is a prerequisite for successful treatment with surgical resection or innovative strategies such as neoadjuvant radiochemotherapy with subsequent orthotopic liver transplantation. Therefore, endoscopic techniques such as cholangioscopy and/or intraductal ultrasound may be useful diagnostic tools in patients with stenoses suspicious for malignancy. Liver Transpl 14: 735-746, 2008.

Surveillance for hepatobiliary cancers in patients with primary sclerosing cholangitis

“…Treatment options and outcomes of patients with HBCa depend to a large extent on tumor stage. Early diagnosis of HBCa is thus imperative for patients to achieve maximal benefit from existing treatment modalities . Because only patients with early‐stage CCA are eligible for liver transplantation (LT) or surgical resection, experts have advocated for surveillance in patients with PSC …”

Primary sclerosing cholangitis in common variable immune deficiency

“…6 This disease can affect the entire biliary tree and lead to fibrosis of the hepatic parenchyma and biliary system, ending in cirrhosis and malignancy. 7 …”

Rethinking the Role of Liver Biopsy in the Era of Personalized Medicine

“…Transhepatic cholangiography can be used if ERCP is unsuccessful, but again is invasive. Non-invasive alternatives to ERCP are: magnetic resonance cholangiopancreatography (MRCP), which is increasingly used and is useful for excluding other disease and evaluating the biliary system [54]. Transient elastography (FibroScan®) has potential as a non-invasive method for detection of cirrhosis in patients with more advanced liver disease [55].…”