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Title: | Epidemiology and outcomes of primary sclerosing cholangitis: an Australian multicentre retrospective cohort study. | Austin Authors: | Tan, Natassia;Ngu, N;Worland, Thomas ;Lee, T ;Abrahams, T ;Pandya, K;Freeman, E;Hannah, N;Gazelakis, K;Madden, R G;Lynch, K D;Valaydon, Z;Sood, Siddharth ;Dev, A;Bell, S;Thompson, A;Ding, J;Nicoll, A J;Liu, K;Gow, P;Lubel, J;Kemp, W;Roberts, S K;Majeed, A | Affiliation: | Gastroenterology Department, Eastern Health, Melbourne, Australia University of Melbourne, Melbourne, Australia Gastroenterology and Hepatology Department, Western Health, Melbourne, Australia Gastroenterology and Hepatology Department, Royal Adelaide Hospital, Adelaide, Australia Gastroenterology and Hepatology Department, The Alfred Hospital, 55 Commercial Rd, Melbourne, 3004, Australia Monash University, Melbourne, Australia Gastroenterology Department, Monash Health, Melbourne, Australia Gastroenterology and Hepatology Gastroenterology and Hepatology Department, St Vincent's Health, Melbourne, Australia AW Morrow Gastroenterology and Liver Centre, Royal Prince Alfred Hospital, Sydney, Australia Gastroenterology and Hepatology Department, Melbourne Health, Melbourne, Australia |
Issue Date: | Oct-2022 | metadata.dc.date: | 2022 | Publication information: | Hepatology International 2022; 16(5): 1094-1104 | Abstract: | Little is known regarding the epidemiology and outcomes of patients with primary sclerosing cholangitis (PSC) in Australia. We, therefore, evaluated the epidemiology and clinical outcomes of PSC in a large cohort of Australian patients and compared these to the general population. We conducted a multicentre, retrospective cohort study of PSC patients at nine tertiary liver centers across three Australian states, including two liver transplant centers. A total of 413 PSC patients with 3,285 person-years of follow-up were included. Three hundred and seventy-one (90%) patients had large duct PSC and 294 (71%) had associated inflammatory bowel disease. A total of 168 (41%) patients developed cirrhosis (including 34 at the time of PSC diagnosis) after a median of 15.8 (95% CI 12.4, NA) years. The composite endpoint of death or liver transplantation occurred in 49 (12%) and 78 (19%) patients, respectively, with a median transplant-free survival of 13.4 (95% CI 12.2-15) years. Compared to the general population, PSC accounted for a 240-fold increased risk of development of cholangiocarcinoma (CCA) and CCA-related death. CCA risk was increased with older age of PSC diagnosis, presence of dominant stricture and colectomy. Compared to same-aged counterparts in the general population, PSC patients who were diagnosed at an older age or with longer disease duration had reduced relative survival. In this large retrospective cohort study of PSC patients in Australia, increased age and time from diagnosis was associated with increased mortality and morbidity particularly from CCA and development of cirrhosis, necessitating need for liver transplant. | URI: | https://ahro.austin.org.au/austinjspui/handle/1/31033 | DOI: | 10.1007/s12072-022-10356-1 | ORCID: | http://orcid.org/0000-0002-0967-3282 | Journal: | Hepatology International | PubMed URL: | 35657479 | Type: | Journal Article | Subjects: | Cholangiocarcinoma Cirrhosis Incidence Liver transplant Malignancy Mortality Primary sclerosing cholangitis Relative survival Risk Transplant-free survival |
Appears in Collections: | Journal articles |
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