![]() ![]() ![]() Hepatomegaly and splenomegaly can be present in 44% and 39% of patients, respectively ( 4, 5). In patients who present with symptoms, abdominal pain is the most frequent symptom (20%) followed by pruritus (10%), jaundice (6%), and fatigue (6%) ( 3), but the presentation may differ widely among patients. In the majority of cases, the diagnosis of PSC is prompted by the finding of cholestasis at the time of routine health evaluation or screening of high-risk patients such as those with inflammatory bowel disease (IBD). PSC is increasingly diagnosed early in the stage of the disease course, and, as a result, the majority of patients do not have any clinical symptoms at the time of diagnosis ( 3). The clinical presentation of PSC correlates with the sequence of inflammatory bile duct destruction and fibrosis, which results in bile duct stricturing, cholestasis, and eventually biliary cirrhosis with end-stage liver disease and hepatic dysfunction ( 2). Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease that is characterized by intra- and/or extrahepatic bile duct injury ( 1). Received: 08 July 2020 Accepted: 19 October 2020 Published: 05 April 2021. Keywords: Cholangiopathy autoimmune liver disease treatment management LT secondary to PSC has an excellent outcome compared to other LT indications, although the disease can recur and result in morbidity post-transplant. LT is currently the only life-extending therapeutic approach for eligible patients with end-stage PSC, ultimately required in approximately 40% of patients. CCA is the most common malignancy in PSC with a cumulative lifetime risk of 10–20%, and accounts for a large proportion of mortality in PSC. ![]() PSC can be complicated by bacterial cholangitis, dominant strictures (DSs), gallbladder polyps and adenocarcinoma, cholangiocarcinoma (CCA) and, in patients with IBD, colorectal malignancy. Management of PSC is mainly focused on treatment of symptoms and addressing complications. PSC is a progressive disease, yet no effective medical therapy for halting disease progression has been identified. The etiology of PSC is poorly understood, but an increasing body of evidence supports the concept of cholangiocyte injury as a result of environmental exposure and an abnormal immune response in genetically susceptible individuals. When symptomatic, the most common presenting symptoms are abdominal pain, pruritus, jaundice or fatigue. PSC is usually diagnosed after detection of cholestasis during health evaluation or screening of patients with IBD. Most cases occur in association with inflammatory bowel disease (IBD), which often precedes the development of PSC. PSC occurs more commonly in men, and is typically diagnosed between the ages of 30 and 40. Policy of Dealing with Allegations of Research MisconductĪbstract: Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic liver disease characterized by inflammatory destruction of the intrahepatic and/or extrahepatic bile ducts, leading to bile stasis, fibrosis, and ultimately to cirrhosis, and often requires liver transplantation (LT).Policy of Screening for Plagiarism Process.
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