Nationwide survey of liver transplantation for Primary Sclerosing Cholangitis in Italy

Introduction Primary Sclerosing Cholangitis (PSC) is a rare condition with low incidence in countries of the Mediterranean Basin, such as Italy; the timing for listing and the indication for liver transplantation (LT) are not entirely stated. Aim To investigate the Italian trend in indication, timing, and outcome for PSC LT. Materials and Methods In April 2022, we conducted a nationwide survey of LT in PSC over the last 15 years. 85% of Italian LT Centers took part in the project. Clinical features, indications and outcomes were evaluated. Results From January 2007 to December 2021, 445 PSC patients have been included in the Italian LT waiting-lists, and 411 underwent LT. The median age at LT was 46 years (18-73), with a high prevalence of males (n=287, 70%). Over 15 years, the number of transplants for PSC increased from 20 to 42 per year, reaching 3.0% of all indications in 2021. Recurring cholangitis and hepatic decompensation were the main indications for listing. The dropout rate from the waiting-list was 6% (26/445), cholangiocarcinoma (CCA) not eligible for LT and being too sick were the leading causes. 71% of the LT Centers utilized induction therapy with Basiliximab. Tacrolimus+MMF was the combined therapy of choice. Overall post-LT mortality was 15% (66/441): 24 patients died in the first year (50% due to surgical complications and 25% infections); 33 patients died between 1 to 5 years (36% PSC recurrence, 21% occult CCA-recurrence) and 9 patients after 5 years (56% cancer, 44% PSC recurrence). Conclusions Likewise other Countries, PSC has been an increasing indication for LT in Italy. The dropout rate from LT waiting-list was relatively low, and CCA not eligible for transplantation or being too sick for surgery were the leading causes. 5-year post-LT survival was 87%, being PSC-recurrence or neoplastic disease the main causes of death.