Epidemiology and outcomes of primary sclerosing cholangitis: an Australian multicentre retrospective cohort study

Abstract

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.

Elevated Liver Fibrosis Progression in Isolated PSC Patients and Increased Malignancy Risk in a PSC-IBD Cohort: A Retrospective Study

“…Similar results were shown in a North American study, where the rate of IBD was significantly lower in older PSC patients [30]. In addition, an Australian multicenter study found a significantly lower rate of liver cirrhosis in patients with PSC and co-occurring IBD compared to patients with isolated PSC [31]. In accordance with these data, we can demonstrate in our study that patients with isolated PSC are diagnosed at a significantly later age compared to PSC patients with concomitant IBD.…”

Primary sclerosing cholangitis in beta-thalassemia with suspected pigment stone: a diagnostic confusion

“…Primary sclerosing cholangitis is a less common disease in South Asia. At the same time, it is more common among males [1,6]. The lack of data about the prevalence and behaviour of primary sclerosing cholangitis in the region can cause a delay in the diagnosis.…”