1. Long-term outcomes of a retrospective multicenter cohort of patients with primary sclerosing cholangitis in Australia: The PROMISE study.
- Author
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Ding J., Nicholl A., Liu K., Gow P., Bell S., Roberts S.K., Majeed A., Ngu N.L.Y., Tan N., Worland T., Lee T., Abrahams T., Pandya K., Dev A., Kemp W., Thompson A., Ding J., Nicholl A., Liu K., Gow P., Bell S., Roberts S.K., Majeed A., Ngu N.L.Y., Tan N., Worland T., Lee T., Abrahams T., Pandya K., Dev A., Kemp W., and Thompson A.
- Abstract
Background and Aim: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with limited effective medical therapies and a paucity of epidemiological and outcome-based data in Australia. We aimed to retrospectively evaluate the demographics and outcomes of an Australian cohort of patients with PSC. Method(s): We performed a retrospective cohort study of patients with PSC at six tertiary health services, including two liver transplant units. Patients were identified by a computerized search of radiological reports. Predictors of transplant-free survival (need for liver transplantation or death from any cause) were analyzed. Result(s): We identified 405 patients with PSC, of whom 261 patients (64.4%) were known to a liver transplant unit. The median age at diagnosis was 36.2 years (IQR, 23.4-50.9), and 261 patients (64.4%) were male. The median alkaline phosphatase (ALP) level at diagnosis was 251 U/L (IQR, 156-436). During a median follow-up of 7.0 years (IQR, 3.3-12.0), the median ALP level improved to 143 U/L (IQR, 92-278) (P < 0.01). Most patients (247, 61%) were prescribed ursodeoxycholic acid (UDCA) at a median dose of 1000 mg (IQR, 750-1000). Of those treated with UDCA, 91 (36.8%) achieved a > 50% reduction in baseline ALP level during follow-up. An overlap syndrome was identified in 47 patients (15.2%), and a dominant stricture (DS) developed in 122 (30.1%). A total of 191 patients (47.3%) developed cirrhosis, of whom 87 (45.6%) had a decompensation episode. There were 282 patients (71.6%) with concomitant inflammatory bowel disease (IBD): ulcerative colitis (54%), Crohn's disease (16%), or IBD unspecified (2%). One hundred patients (24.7%) underwent liver transplantation, and 51 (12.6%) died (82.3% liver-related death). Gastrointestinal malignancies included cholangiocarcinoma (CCA) (26, 6.4%), hepatocellular carcinoma (HCC) (12, 3.0%), and colorectal cancer (15, 3.8%). The proportion of transplant-free survival was 88.5% at 5 years, 73.8%
- Published
- 2021