Please use this identifier to cite or link to this item: https://ahro.austin.org.au/austinjspui/handle/1/30277
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, Paul J ;Lubel, J;Kemp, W;Roberts, S K;Majeed, A
Affiliation: Gastroenterology Department, Monash Health, Melbourne, Australia..
Gastroenterology Department, Eastern Health, Melbourne, Australia..
Monash University, Melbourne, Australia..
Gastroenterology and Hepatology Department, The Alfred Hospital, 55 Commercial Rd, Melbourne, 3004, Australia..
Monash University, Melbourne, Australia..
AW Morrow Gastroenterology and Liver Centre, Royal Prince Alfred Hospital, Sydney, Australia..
Gastroenterology and Hepatology
University of Melbourne, Melbourne, Australia..
Gastroenterology and Hepatology Department, St Vincent's Health, Melbourne, Australia..
Gastroenterology and Hepatology Department, Melbourne Health, Melbourne, Australia..
Gastroenterology and Hepatology Department, Western Health, Melbourne, Australia..
Gastroenterology and Hepatology Department, Royal Adelaide Hospital, Adelaide, Australia..
Issue Date: 3-Jun-2022
metadata.dc.date: 2022
Publication information: Hepatology international 2022; online first: 3 June
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/1/30277
DOI: 10.1007/s12072-022-10356-1
ORCID: http://orcid.org/0000-0002-0967-3282
http://orcid.org/0000-0001-5122-5750
http://orcid.org/0000-0002-9341-4792
http://orcid.org/0000-0001-6505-7233
PubMed URL: 35657479
PubMed URL: https://pubmed.ncbi.nlm.nih.gov/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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