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P202. Primary sclerosing cholangitis: A case series and natural history

H. Yanai1, S. Matalon1, N. Fliss Isakov1, E. Santo1, I. Dotan2

1Tel Aviv Sourasky Medical Center, Department of Gastroeneterology and Liver Diseases, Tel Aviv, Israel; 2Tel Aviv Sourasky Medical Center, Department of Gastroenterology and Liver Diseases, Tel Aviv, Israel

Background: Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic liver disorder often associated with inflammatory bowel diseases (IBD) and carries a grave prognosis. We aimed to assess the clinical characteristics and natural course of PSC in a tertiary referral center.

Methods: A retrospective study was performed using chart review of PSC patients seen at the Tel Aviv Sourasky Medical Center between 1997–2011. Clinical characteristics, laboratory and imaging data were correlated with outcome.

Results: PSC was confirmed in 68 patients, 38 males (56%), average age at presentation 37±2.0 (12–77) years. Follow-up duration was 5.42±0.8 years. Sixty percent were Ashkenazi, 24.2% Sephardic and 15.2% non-Jews. Most patients were non smokers (88%). Forty six patients had IBD (69.6% ulcerative colitis [UC], 28.2% Crohn's Disease [CD] and 2.2% IBD-unclassified). Nearly 90% of UC patients had pancolitis, and 60% of the CD patients had ileal involvement. MRCP was the most frequently used diagnostic modality (70.7%), while in 36% more than one diagnostic tool was required to establish the diagnosis. About half of the patients had both intra and extra-hepatic involvement, 39.4% had only intra-hepatic disease and 3% – isolated small duct disease. Three patients fulfilled the criteria for overlap syndrome. At the end of follow-up, 4 patients had undergone liver transplantation, and another 13 had cirrhosis. Three patients were diagnosed with cholangio-carcinoma, and 3 others were diagnosed with colon cancer. All patients in this cohort were treated with ursodeoxycholic acid and 50.5% of the IBD patients were treated with mesalamine derivatives as well. PSC patients with IBD were more likely to be symptomatic at presentation contrasting patients with isolated PSC, who were occasionally diagnosed based on abnormal laboratory results only (p = 0.039). Nonetheless, laboratory findings such as elevated liver enzymes as well as clinical outcome did not differ between these groups. Intrahepatic involvement was more common in CD contrasting UC patients (p = 0.05), as were features of autoimmune hepatitis, however prognosis was comparable.

Conclusions: PSC has a dismal prognosis with end stage liver disease and/or malignancy complicating more than a third of the patients in this cohort. Outcome appears to be similar in both IBD and non IBD as well as among UC and CD. Identifying better predictors of progression is needed.