Management of Inflammatory Bowel Disease Associated With Primary Sclerosing Cholangitis Pre- and Post-liver...

Primary sclerosing cholangitis (PSC)-associated inflammatory bowel disease (IBD) is considered a distinct IBD phenotype, with probably a distinct pathogenesis according to genome-wide association studies and mucosal immunology and microbiome studies. Management of IBD in patients with PSC presents with specific monitoring and therapeutic challenges. Both IBD-related and treatment-related complications have to be carefully considered in the context of hepatobiliary disease, end-stage liver disease, and liver transplant recipients. Standard IBD drug therapies are generally effective and well-tolerated in PSC-IBD, both pre- and post-liver transplantation. However, evidence of direct benefit on PSC progression is limited and largely based on surrogate endpoints such as alkaline phosphatase reduction. Vedolizumab and adalimumab have shown modest biochemical improvements in selected cohorts, although no therapy has demonstrated consistent disease-modifying effects on PSC. Based on current available data, vancomycin seems promising to improve IBD and PSC outcomes. In patients with an indication for colectomy, counseling on functional outcome in general and on disease-specific risks of recurrent intestinal inflammation, neoplasia, and PSC progression is warranted. Immunosuppressive regimens posttransplant and persistent intestinal inflammation influence graft survival and PSC recurrence. Thus, management in patients with PSC and IBD requires a tailored, risk-adapted approach, integrating control of intestinal inflammation, hepatobiliary monitoring, and cancer surveillance. This contemporary review aimede to elucidate the current understanding regarding the efficacy and safety of medical and surgical therapies used for IBD in light of the pre- and posttransplant course of patients with PSC-IBD.