Liver transplantation for cholestatic liver diseases: Trends in primary biliary cholangitis and primary sclerosing cholangitis in the US and Europe over two decades.

Cholestatic liver diseases [primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC)] are important indications for liver transplantation (LT). Adult LT recipients with PBC or PSC were identified from the European Liver Transplant Registry (ELTR) and U.S. Scientific Registry of Transplant Recipients (SRTR) (2000-2022). We assessed temporal trends in PBC/PSC prevalence among the LT population and used Cox models to identify variables associated with post-transplant mortality in PBC/PSC. A total of 22,034 LT recipients were included (PBC n=8774; PSC n=13,260). HCC was present in 5.4% PBC, and cholangiocarcinoma in 2.4% PSC. PBC recipients had more ascites and hepatic encephalopathy, higher MELD scores (20±9 vs. 19±9) ( p <0.0001). PBC as a proportion of all LTs declined from 6.2% (2000) to 2.9% (2022) ( p <0.0001), while PSC remained stable (5.9% to 5.7%, p >0.05). The declines in PBC-LTs were observed in both SRTR and ELTR; PSC has shown a recent increase in ELTR: 4.9% to 7.3% ( p <0.05). Post-transplant recipient survival for PBC: 1-year 90%, 3-year 85%, 5-year 81%, and 10-year 68%; for PSC: 1-year 92%, 3-year 86%, 5-year 81%, and 10-year 69%. In multivariable survival analyses, post-transplant mortality in PBC was independently associated with earlier calendar year, older age, male sex, hepatic encephalopathy, HCC, and higher donor age (all p <0.05). In PSC, post-transplant mortality was also associated with earlier calendar year, older age, male sex, higher MELD score, presence of cholangiocarcinoma, receiving LT in a small transplant center, and older donor's age (all p <0.05). Liver transplantations for PBC have declined, which may reflect improved medical therapy; PSC has remained stable, with recent increases in Europe. Three-year post-transplant survival exceeds 85% for both groups and continues to improve.