Abstract

Though rare, primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) account for 8-14% of liver transplants (LT) in North America and Europe and the journey of these patients across the peri-transplant period is unique. Equitable access to LT is an important challenge, as the MELD score and its derivatives inadequately reflect the morbidity and mortality related to these diseases failing to capture disease-specific complications, such as recurrent cholangitis, malignancy risk, severe portal hypertension, and sarcopenia. The waitlist experience is high-risk, prolonged and a distinct form of "MELD purgatory". Once barriers to access are overcome, post-transplant outcomes are generally excellent, however disease recurrence affects 15-35% at 5 to 10 years post-LT with increasing rates over time. Diagnosing recurrence is challenged by a broad differential for post-transplant biliary injury, and the risk factors for its development remain controversial. While post-LT use of ursodeoxycholic acid in PBC is clearly beneficial, no effective medical therapy currently exists for recurrent PSC. A heightened focus on control of inflammatory bowel disease activity is critical as a potentially important modifiable risk factor for rPSC, including with escalation of medical therapy as needed and timely colectomy when indicated. This review outlines the journey for patients with PBC and PSC, from transplant listing to post-transplant management, emphasizing the need for unique and tailored approaches to optimize outcomes and long-term survival.