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EASL Congress
April 10, 2014
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Primary sclerosing cholangitis risk parameters identified among international cohort

LONDON — International study results showed risk parameters that influenced the clinical course of primary sclerosing cholangitis, according to research presented at the International Liver Congress.

Tobias J. Weismüller, MD, department of internal medicine, University of Bonn, Germany, and fellow researchers studied data on 8,212 patients diagnosed with primary sclerosing cholangitis (PSC) between 1980 and 2010 at 33 institutions in 16 countries, which were divided into six geographical regions. One-third of the patients were from the United States. Retrospective analysis was conducted on clinical presentation, survival, liver transplantation (LT), inflammatory bowel disease (IBD) and malignancy data. Mean follow-up was 21 years.

There were 6,205 patients (mean age at diagnosis, 40 years; 65.1% men) included in the final analysis. Large-duct PSC was experienced by 89% of patients, small-duct PSC by 4% of patients, and PSC with features of autoimmune hepatitis (AIH) in 6.4%. Sixty-nine percent of patients had IBD (79.5% ulcerative colitis). Transplant-free survival rates at 5, 10 and 20 years and overall survival were 80.7%, 72%, 66.3% and 65.6%, respectively. Median transplant-free survival was 14.5 years.

Diagnoses of hepatobiliary malignancies occurred in 10.9% of patients and colorectal carcinoma in 11.2% of patients. A reduced incidence of hepatobiliary malignancies was experienced by women (P<.001), who also had a higher transplant-free survival rate (P=.045). Better transplant-free survival and a lower rate of hepatobiliary malignancies were observed in patients with small duct disease and with AIH features (P<.001). IBD had an insignificant influence on survival and malignancy rate.

“This multicentric study-approach avoids influence of center-specific and regional characteristics and identifies risk parameters that obviously moderate the clinical course of primary sclerosing cholangitis,” Weismüller said at his presentation. – Bruce Thiel

Disclosure: Weismüller reported no relevant financial disclosures.

For more information:

Weismüller TJ. #05: Primary Sclerosing Cholangitis from a Global Perspective — A Multicenter, Retrospective, Observational Study of the International PSC Study Group. Presented at: The International Liver Congress 2014; April 9-13, London.