Primary Sclerosing Cholangitis: Diagnosis, Prognosis, and Management

Abstract

Primary sclerosing cholangitis (PSC) is a chronic immune-mediated disease of the liver of unclear etiology, characterized by chronic inflammation and fibrosis of bile ducts. It primarily affects middle aged men, and is associated with 4-fold increased mortality as compared to ageand gender-matched population. Progressive biliary and hepatic damage results in portal hypertension and hepatic failure in a significant majority of patients over a 10–15 year period from initial diagnosis. In addition, PSC confers a markedly increased risk of hepatobiliary cancer, including cholangiocarcinoma and gallbladder cancer as compared to the general population, and cancer is the leading cause of mortality in patients with PSC. It is associated with inflammatory bowel disease in 70% patients, and increases the risk of colorectal cancer almost 10-fold. Despite significant research efforts in this field, the pathogenic mechanisms of PSC are still incompletely understood, although growing evidence supports the role of genetic and immunologic factors. There are no proven medical therapies that alter the natural course of the disease. Thus, liver transplantation is the only available treatment for patients with advanced PSC, with excellent outcomes in this population.

Extra-intestinal malignancies in inflammatory bowel diseases: An update with emphasis on MDCT and MR imaging features

“…The diagnosis is suggested by the presence of altered liver function tests. This form in association with IBD is observed in 15% of patients, has a better long-term survival rate and carries a low risk for cholangiocarcinoma [25,37]. On the opposite, PSC may involve larger bile ducts, including intrahepatic ones and biliary confluence, and is at high risk of malignant transformation [38].…”

“…Primary sclerosing cholangitis (PSC), which is considered as an intermediate step in the development of cholangiocarcinoma is associated with IBD in 60 to 80% of patients [25]. UC is present in 48 to 86% of patients and CD in 13 to 25% [25].…”

“…UC is present in 48 to 86% of patients and CD in 13 to 25% [25]. Conversely, PSC is the most common hepatobiliary complication observed in IBD patients and seen in approximately 5% of IBD patients [14,[25][26][27].…”

“…is almost always associated with IBD [14]. Of major importance, the risk for cholangiocarcinoma is dramatically more marked in IBD patients with PSC, which is 160-fold that of the general population and estimated to a 5% -10% lifelong risk [25]. The peak incidence of cholangiocarcinoma is observed during the first years following the diagnosis of IBD [14].…”

Gender Mainstreaming and Transplant Surgery

“…It is suspected that women are allocated for smaller and older organs associated with worse graft function, which does not affect survival [15,35]. In conclusion, the outcome for both sexes after transplantation is equal for all indications except hepatitis C-induced cirrhosis [4,15,28,36,37,38,39,40,41,42,43]. Women undergoing transplantation because of a hepatitis C-induced cirrhosis have a higher risk for graft loss and rejection and a worse outcome in the case of a recurrent hepatitis C infection [8,15,44,45,46].…”