Kidney-liver transplantation and liver transplantation in autosomal dominant polycystic kidney disease: new diagnostic and clinical assessment tools

Background: ADPKD is a leading cause of ESKD. Severe hepatic involvement (3-8% of patients) may necessitate liver transplantation (LT) or combined liver-kidney transplantation (CLKT). However, the optimal strategy to preserve renal function and maximize patient survival remains a key clinical question. Aims and Methods: A retrospective cohort study of 54 ADPKD patients who received either CLKT (n=39) or isolated LT (n=15) (01/2004 - 07/2024). A control group of 78 matched isolated kidney transplant (KT) recipients was included.Endpoints included renal survival (freedom from renal replacement therapy, RRT), progression of renal dysfunction, kidney graft rejection, eGFR slope (linear mixed-effects models), and overall patient survival. Results: CLKT patients had advanced baseline kidney disease (65% on dialysis), while LT patients had preserved function. Genetic testing mainly identified PKD1 variants. Over a mean 7.5-year follow-up, CLKT renal survival was superior to LT; the latter experienced significant renal decline (slope -3.24 ml/min/year vs -0.012 in CLKT). Compared to matched KT, CLKT showed a trend towards superior 10-year RRT-free survival (p=0.051), slower renal decline (p=0.015), and fewer acute rejections (p=0.011). Longitudinal eGFR analysis confirmed these findings, showing a stable slope in the CLKT group (-0.012 ml/min/year), in contrast to a more rapid decline in the LT group (-3.24 ml/min/year) and a moderate decline in the KT group (-0.84 ml/min/year). Overall survival at 1, 5, and 10 years was 84.6% for CLKT, 98.7% for KT, and 93.3%, 93.3%, and 74.6% for LT. Conclusion: A comprehensive, multidisciplinary patient evaluation is crucial for tailoring the optimal transplant strategy. Integrating genetic profiling with volumetric assessment of liver and kidneys (TLV/TKV) and renal function analysis allows for a highly effective, tailored transplantation strategy in ADPKD. Adopting this approach to patient selection enables clinicians to identify candidates most likely to achieve favorable long-term success, strongly supporting an organ-sparing strategy.