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

BACKGROUND: Cholestatic liver diseases [primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC)] are important indications for liver transplantation (LT). METHODS: 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 LT population and used Cox models to identify variables associated with post-transplant mortality in PBC/PSC. RESULTS: A total of 22,034 LT recipients were included (PBC n=8,774; PSC n=13,260). HCC was present in 5.4% PBC, 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%, 10-year 68%; for PSC: 1-year 92%, 3-year 86%, 5-year 81%, 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). CONCLUSIONS: Liver transplantations for PBC have declined which may reflect improved medical therapy; PSC have remained stable, with recent increases in Europe. Three-year post-transplant survival exceeds 85% for both groups and continues to improve.