J Hepatol. 2023 Nov 6:S0168-8278(23)05158-9. doi: 10.1016/j.jhep.2023.09.031.Online ahead of print.

Kim N van Munster ¹, Annika Bergquist ², Cyriel Y Ponsioen ³

Author information

¹Department of Gastroenterology and Hepatology, Amsterdam UMC, University of Amsterdam, the Netherlands.

²Department of Medicine Huddinge, Division of Hepatology, Karolinska Institutet, Department of Upper GI Disease, Karolinska University Hospital, Stockholm, Sweden.

³Department of Gastroenterology and Hepatology, Amsterdam UMC, University of Amsterdam, the Netherlands. Electronic address: c.y.ponsioen@amsterdamumc.nl.

Abstract

Primary sclerosing cholangitis (PSC) was declared one of the biggest unmet needs in hepatology during ILC 2016 in Berlin. Since then, not much has changed unfortunately, largely due to the still elusive pathophysiology of the disease. One of the most striking features of PSC is its association with inflammatory bowel disease (IBD), with the majority of patients with PSC being diagnosed with extensive colitis. This review describes the epidemiology of IBD in PSC, its specific phenotype, complications and potential pathophysiological mechanisms connecting the two diseases. Whether PSC is merely an extra-intestinal manifestation of IBD or if PSC and IBD are two distinct diseases that happen to share a common susceptibility that leads to a dual phenotype is debated. Implications for the management of the two diseases together are also discussed. Overall, this review summarises the available data in PSC-IBD and discusses whether PSC and IBD are one or two disease(s).