Primary Sclerosing Cholangitis Overlapping with IBD

摘要: Primary sclerosing cholangitis is a chronic and progressive cholestatic disease, characterized by inflammation fibrosis of the intrahepatic and/or extrahepatic ducts, that may result in liver cirrhosis eventually end-stage disease. No medical treatment available, transplantation remains only curative option, albeit with an elevated recurrence rate. Having diagnosis inflammatory bowel disease strongest risk factor for PSC development, since 70% patients have underlying IBD, most frequently ulcerative colitis. For unknown reasons, coexistence IBD seems to modify phenotype course. PSC-IBD typically extensive colonic involvement, mild activity symptoms, rectal sparing, backwash ileitis, increased developing pouchitis after proctocolectomy. Furthermore, some studies suggest there exist inverse relationship between severity activity. Importantly, present very high colorectal neoplasia, usually located right colon, requiring routine endoscopic surveillance (preferably using chromoendoscopy) every year, starting from moment diagnosed.