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.