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A contribution to the differential diagnostics of sclerosing cholangitides


Authors: Martin Blaho;  Petr Dítě;  Martina Bojková;  Martin Rydlo;  Tomáš Kupka;  Pavel Svoboda;  Pavel Klvaňa;  Arnošt Martínek
Authors‘ workplace: Gastroenterologické oddělení Interní kliniky LF OU a FN Ostrava
Published in: Vnitř Lék 2017; 63(1): 50-55
Category: Reviews

Overview

Sclerosing cholangitides represent a group of chronic biliary obstructive diseases which include primary sclerosing cholangitis (PSC), IgG4 associated sclerosing cholangitis (IgG4-SC) and secondary sclerosing cholangitis (SSC). The manifestations of the diseases are similar, but their asymptomatic course is also frequent. IgG4-SC belongs to the group of IgG4 associated diseases and it is the most frequently related to type 1 autoimmune pancreatitis. Diagnosing of IgG4-SC is based on typical histopathological images, shape changes revealed by diagnostic imaging, serological tests, concurrent impairment of other organs and response to therapy, where IgG4-SC responds well to treatment with corticoids, whereas the only possibility for the remaining units is endoscopic intervention or liver transplantation. Secondary sclerosing cholangitis may develop as a result of many different insults affecting the biliary tree. Among them, the most frequently described include long-lasting biliary obstruction, surgical injury of the biliary tree, and ischemic cholangitis in liver allotransplants or recurrent pancreatitis. We use serological and imaging examination in PSC diagnostics, sometimes we have to resort to liver biopsy. PSC is to a significant degree accompanied by the presence of idiopathic bowel disease, typically ulcerative colitis. As a result, PSC may lead to cirrhosis of the liver and it is a precancerous condition of several malignancies. With regard to variable locations of the biliary tree injuries concerning the aforementioned units, also certain malignancies in subhepatic landscape need to be considered in the differential diagnosis: pancreatic cancer and cholangiogenous carcinoma.

Key words:
genetic factors – IBD – IgG4 cholangitis – liver transplantation – bile duct cancer – ursodeoxycholic acid – primary sclerosing cholangitis – secondary cholangitis – sclerosing cholangitis


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