Epidemiology and outcomes of primary sclerosing cholangitis with and without inflammatory bowel disease in an Australian cohort

Abstract

Australian PSC patients have similar characteristics compared to European and North American cohorts. IBD is a significant predictor of gastrointestinal malignancies. Deaths were more common in PSC-IBD but overall transplant-free survival remained similar in PSC-IBD and PSC-only groups. An elevated INR was an independent predictor of death or liver transplantation.

Long‐term outcomes of primary sclerosing cholangitis: an Australian non‐transplant tertiary hospital perspective

“…First, although being a large academic liver centre, our hospital does not include a liver transplantation service, and as such may not be referred patients with more severe, end‐stage disease. Only one (3%) patient in our series underwent a liver transplantation (in another hospital), compared to 8–40% in other studies and this may overestimate the survival rate. Additionally, the median follow‐up time in our study was 5.5 years, which is at the lower end of the 5.2–9.6 years reported in other studies .…”

“…Only one (3%) patient in our series underwent a liver transplantation (in another hospital), compared to 8–40% in other studies and this may overestimate the survival rate. Additionally, the median follow‐up time in our study was 5.5 years, which is at the lower end of the 5.2–9.6 years reported in other studies . Still, only four (10%) patients were lost to follow up, meaning that complications were captured in most patients during the study period.…”

“…Although our patients’ baselines characteristics were similar to those of the 2017 Australian study of 208 PSC patients in terms of age and gender, far fewer of our cohort reached the primary end‐points of death or liver transplantation (21% vs 41%) or developed gastrointestinal malignancy (8% vs 17%). This may be explained by our smaller proportion of cirrhotic patients (38% vs 50%) and a higher proportion of those with small‐duct PSC (14% vs 5%), although there was in fact no survival difference between those with or without small‐duct disease.…”

“…PSC has been studied in many different cohorts worldwide, but to date there have been limited published Australian data, with the only study available predominantly being from a quaternary liver transplant centre that is subject to potential referral bias . We therefore performed a comprehensive, retrospective audit of patients with PSC at The Alfred Hospital, a large metropolitan non‐transplant teaching hospital in Melbourne, Australia, to evaluate the morbidity and mortality in this group of patients and to assess the degree of disease progression over time.…”

Geoepidemiologic variation in outcomes of primary sclerosing cholangitis

“…Moreover, geographic variations may exist in the PSC-IBD phenotype. In particular, studies from Oceania have noted patients with PSC-IBD to be at an increased risk of death and increased risk of gastrointestinal or hepatobiliary malignancies than patients with PSC alone[ 45 , 46 ]. In contrast, multiple studies from Asia have not identified significant differences in these outcome measures between patients with PSC-IBD and PSC alone[ 16 , 17 ].…”