Occasional patients with recurrent bacterial cholangitis may benefit from prophylactic long term antibiotics. Rarely, recurrent cholangitis can be so severe as to become the primary indication for OLT.67 The management of pruritus in PSC patients should prompt consideration of a dominant stricture. In the absence of a dominant stricture, the management of pruritus is similar to that for pruritus in primary biliary cirrhosis. Please see the AASLD Guidelines on the Erastin purchase Management of PBC.68 Recommendations: 6 In patients with increases in serum bilirubin and/or worsening
pruritus progressive bile duct dilatation on imaging studies, and/or cholangitis, we recommend performing an ERC promptly to exclude a dominant stricture (1B). When cirrhosis is present in a patient with PSC, portal hypertension (PHT) will gradually develop because it constitutes part of the natural history of all patients with cirrhosis. As is the case with cirrhosis in general, the platelet count is a predictor of the presence of esophageal varices Tamoxifen ic50 also in PSC. Of 283 PSC patients newly diagnosed at Mayo Clinic (Rochester, MN) 36% (n = 102) had varices, of which 56% had moderate/large varices and 28 had already bled. Independent predictors of esophageal varices and of moderate/large
size varices were platelet count, albumin level, and advanced histologic disease. After controlling for the presence of advanced histologic stage and albumin levels, the odds ratios (OR) of platelet count less than 150 × 103/dL for the presence of esophageal varices was 6.3 (95% CI: 2.6–15.8).69 Similar conclusions were reached in Mexico in a smaller group.70 Portal hypertension
may, however, be present in patients with PSC who do not yet have cirrhosis, but this is uncommon. Of 306 liver transplants performed during 1995–2003 for chronic biliary tract disease, 26 (8.5%) underwent OLT in the precirrhotic selleckchem stage; 18 of them had PSC. Of the 11 patients with portal hypertension as the major indication for OLT, nodular regenerative hyperplasia (NRH) was prominent in 8 (73%) and obliterative portal venopathy in 6 (55%) at histopathological examination. Thus, precirrhotic PHT may contribute as an indication for OLT.67 The management of portal hypertension in patients with PSC does not differ from non-PSC patients and has been discussed in prior AASLD guidelines.71 Hepatic osteodystrophy is the term used for the metabolic bone disorders associated with chronic liver diseases. The diagnosis is made by bone mineral density measurement whereby osteopenia is characterized by a T-score between 1 and 2.5 standard deviations below the density observed in young normal individuals, and osteoporosis as a T-score beneath 2.5. The incidence of osteoporosis in PSC is between 4 and 10%. The incidence increases with decreasing body mass index, with increasing duration of the disease, with age, and possibly with severity of the disease,72 although this was not confirmed.