Treatment of primary sclerosing cholangitis combined with inflammatory bowel disease

Abstract

Primary sclerosing cholangitis (PSC) is a progressive cholestatic, inflammatory, and fibrotic disease that is strongly associated with inflammatory bowel disease (IBD). PSC-IBD represents a unique disease entity and patients with this disease have an increased risk of malignancy development, such as colorectal cancer and cholangiocarcinoma. The pathogenesis of PSC-IBD involves genetic and environmental factors such as gut dysbiosis and bile acids alteration. However, despite the advancement of disease characteristics, no effective medical therapy has proven to have a significant impact on the prognosis of PSC. The treatment options for patients with PSC-IBD do not differ from those for patients with PSC alone. Potential candidate drugs have been developed based on the pathogenesis of PSC-IBD, such as those that target modulation of bile acids, inflammation, fibrosis, and gut dysbiosis. In this review, we summarize the current medical treatments for PSC-IBD and the status of new emerging therapeutic agents.

Keywords: Inflammatory bowel disease; Primary sclerosing cholangitis; Therapeutics.

Fig. 1.

Therapeutic approach of primary sclerosing cholangitis (PSC) combined with inflammatory bowel disease (IBD). IBD management should be continued regardless of PSC treatment. Symptomatic care and management of complications of PSC are currently implemented, however, with the advancement of understanding of pathogenesis of PSC-IBD, several new therapeutic agents are developed and undergone the clinical trials. UDCA, ursodeoxycholic acid; TPL, transplantation; norUDCA, norursodeoxycholic acid; FXR, farnesoid X receptor; PPARs, peroxisome proliferator-activated receptors; ASBT, apical sodium-dependent bile acid transporter; LOXL2, lysyl oxidase-like 2; CCR2, C-C chemokine receptor 2; CCR5, C-C chemokine receptor 5; α4β7, alpha 4 beta 7; VAP-1, vascular adhesion protein 1; TNF, tumor necrosis factor.