Please use this identifier to cite or link to this item: https://ahro.austin.org.au/austinjspui/handle/1/31033
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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