Primary sclerosing cholangitis: Summary of a workshop

Abstract

Primary sclerosing cholangitis (PSC) is a rare but important liver disease that leads to cirrhosis and need for liver transplantation in a high proportion of cases. The disease occurs in approximately 1 per 100,000 population per year, usually presents in adulthood, and affects men more often than women. Typical serum biochemical results, autoantibodies and liver biopsy are suggestive but not diagnostic of PSC, the diagnosis requiring cholangiographic demonstration of stricturing and dilatation of the intra-and/or extra-hepatic bile ducts. The natural history of PSC is variable, the average survival being 12 to 17 years. The cause of PSC is still unknown. Although considered an autoimmune disease, PSC has several atypical features and a strong genetic component. The therapy of PSC is unsatisfactory. Standard doses of ursodeoxycholic acid (UDCA) lead to improvements in biochemical abnormalities but not in histology, cholangiographic appearance or survival. Several innovative therapies have been tried in PSC, but with scant evidence of benefit. For patients with high grade strictures, endoscopic dilatation is beneficial. Liver transplantation is successful for end-stage liver disease due to PSC and improves survival. PSC may recur after transplantation but is rarely progressive. The most dreaded complication of PSC is cholangiocarcinoma. Diagnosis of this highly malignant tumor is difficult, and there are no biomarkers for its early detection. Liver transplantation for cholangiocarcinoma has an exceedingly poor outcome, although transplantation with neoadjuvant chemoirradiation holds promise in selected patients. Thus, significant opportunities remain for basic and clinical research into the cause, natural history, and therapy of PSC. 44:746-764.)

How Our Practice of Histopathology, Especially Tumour Pathology has Changed in the Last Two Decades: Reflections from a Major Referral Center in Pakistan

“…Since the mid-nineties, we have used the Scheuer system (Scheuer, 1991) for grading and staging of hepatitis in liver biopsies. During the last five to seven years, we have also begun to report such diverse entities like primary sclerosing cholangitis (LaRusso et al, 2006), extra hepatic biliary atresia (Sokol et al, 2007), congenital hepatic fibrosis (Desmet, 1992), neonatal hepatitis (Ballistreri and Bezerra, 2006), nonalcoholic steato hepatitis (NASH) (Ludwig et al, 1980) etc. Metastatic carcinomas are commonly seen in our trucut liver biopsies, and cytokeratins 7 and 20 and other more specific IHC markers supplement the histologic features and help us in determining the probable sites of origin.…”

The Value of Molecular Biomarkers in Biliary Tract Cancer in the Era of Targeted Therapy

“…6 The potential survival benefit of early detection is obvious, with survival of 70-80% for patients with incidentally detected early CC on liver transplantation for PSC. 7 The value of tumor markers for biliary cancers lies not only in trying to achieve an early diagnosis but also in decision making for institution of toxic and expensive chemotherapy or for undertaking of radical surgery, both of which may be futile if the disease is too advanced. A molecular marker from serum samples or from biopsy may be able to predict tumor behavior and may help in the decision-making process in today's era of targeted therapy.…”

Recurrence of cholestatic liver disease after living donor liver transplantation

“…UDCA administration, with or without the use of immunosuppressive agents, seems to have a beneficial effect on liver function tests, assessed by blood chemistries. But, it is not clear whether there is a beneficial effect on delaying the progression of the disease and improving the overall survival rate [8] . Liver transplantation is the optimal treatment for patients with PBC or PSC presenting with ESLD and associated complications, such as severe manifestations of portal hypertension [5,8] .…”

“…But, it is not clear whether there is a beneficial effect on delaying the progression of the disease and improving the overall survival rate [8] . Liver transplantation is the optimal treatment for patients with PBC or PSC presenting with ESLD and associated complications, such as severe manifestations of portal hypertension [5,8] . The short-term mortality rate, due to disease recurrence following transplantation, is lower in patients with PBC or PSC than in those with hepatitis C or hepatic malignancy.…”