Liver transplantation for cholestatic liver diseases: Timing and disease recurrence

Though rare, primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) account for 8%-14% of liver transplants (LTs) 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, posttransplant outcomes are generally excellent; however, disease recurrence affects 15%-35% at 5-10 years after LT with increasing rates over time. Diagnosing recurrence is challenged by a broad differential for posttransplant 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 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 posttransplant management, emphasizing the need for unique and tailored approaches to optimize outcomes and long-term survival.