TY - JOUR
T1 - Epidemiology and outcomes of primary sclerosing cholangitis
T2 - an Australian multicentre retrospective cohort study
AU - Tan, Natassia
AU - Ngu, N.
AU - Worland, T.
AU - Lee, T.
AU - Abrahams, T.
AU - Pandya, K.
AU - Freeman, E.
AU - Hannah, N.
AU - Gazelakis, K.
AU - Madden, R. G.
AU - Lynch, K. D.
AU - Valaydon, Z.
AU - Sood, S.
AU - Dev, A.
AU - Bell, S.
AU - Thompson, A.
AU - Ding, J.
AU - Nicoll, A. J.
AU - Liu, K.
AU - Gow, P.
AU - Lubel, J.
AU - Kemp, W.
AU - Roberts, S. K.
AU - Majeed, A.
AU - the Melbourne Liver Group
N1 - Funding Information:
Open Access funding enabled and organized by CAUL and its Member Institutions. This study was funded by the Alfred Hospital Department of Gastroenterology and an Australian Government Research Training Program Scholarship. The study was supported by all contributing study centers. Financial support was also obtained from Dr Falk Pharma who had no influence on data acquisition, analyses performed or drafting of the manuscript.
Publisher Copyright:
© 2022, The Author(s).
PY - 2022/10
Y1 - 2022/10
N2 - Background and aims: 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. Methods: We conducted a multicentre, retrospective cohort study of PSC patients at nine tertiary liver centers across three Australian states, including two liver transplant centers. Results: 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. Conclusion: 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.
AB - Background and aims: 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. Methods: We conducted a multicentre, retrospective cohort study of PSC patients at nine tertiary liver centers across three Australian states, including two liver transplant centers. Results: 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. Conclusion: 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.
KW - Cholangiocarcinoma
KW - Cirrhosis
KW - Incidence
KW - Liver transplant
KW - Malignancy
KW - Mortality
KW - Primary sclerosing cholangitis
KW - Relative survival
KW - Risk
KW - Transplant-free survival
UR - http://www.scopus.com/inward/record.url?scp=85133878391&partnerID=8YFLogxK
U2 - 10.1007/s12072-022-10356-1
DO - 10.1007/s12072-022-10356-1
M3 - Article
C2 - 35657479
AN - SCOPUS:85133878391
SN - 1936-0533
VL - 16
SP - 1094
EP - 1104
JO - Hepatology International
JF - Hepatology International
IS - 5
ER -