Association of autosomal dominant polycystic kidney disease (ADPKD) with cardiovascular disease and mortality in patients with treated kidney failure.

Abstract

Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is a multisystem syndrome associated with significant morbidity and mortality, particularly kidney failure. This study sought to evaluate mortality and transplant outcomes in Australian and New Zealand patients with ADPKD commencing kidney replacement therapy (KRT).

Methods: A retrospective review of all patients with kidney failure over 18 years of age commencing KRT between 1963 and 2020, stratified across eras, using Australia and New Zealand Transplant (ANZDATA) Registry data. People with ADPKD were compared to those with other causes of kidney failure (non-ADPKD). The primary outcome was mortality on dialysis. Secondary outcomes included post-kidney transplant patient survival and graft survival (both death-censored and with death as a competing risk). Outcomes were compared using multivariable Cox proportional hazards models.

Results: During the study period, 63,875 patients commenced KRT, including 2,466 (3.9%) with ADPKD. Compared to patients without ADPKD, those with ADPKD had generally fewer comorbidities at KRT initiation. ADPKD was independently associated with a lower risk of mortality on dialysis (adjusted hazard ratio [aHR], 0.71, 95% CI 0.67-0.75, p < 0.001). Causes of death were similar between the ADPKD and non-ADPKD cohorts including for cardiovascular disease (27.5% vs. 27.6%, respectively), infection (7.9% vs. 8.5%) and cancer (3.4% vs. 3.5%). Kidney transplant recipients had fewer comorbidities across both cohorts, with a similar incidence of coronary artery disease (ADPKD 7.2% vs. non-ADPKD 8.0%). Compared with non-ADPKD, ADPKD was associated with a similar mortality risk in kidney transplant recipients (aHR, 0.96, 95% CI 0.88-1.05) and slightly improved graft survival (aHR, 0.87; 95% CI 0.77-0.97, p < 0.05), although there was no difference in death-censored graft survival (aHR, 0.92, 95% CI 0.79-1.07).

Conclusions: Compared to other causes of kidney failure, ADPKD is associated with better patient survival on dialysis, similar patient survival post-kidney transplantation and similar death-censored graft survival.