Nationwide epidemiological survey of autosomal dominant polycystic kidney disease in Japan in 2022.

Abstract

Accurate epidemiological data on autosomal dominant polycystic kidney disease (ADPKD) are essential for healthcare planning and therapeutic strategies. However, nationwide prevalence estimates in Japan have not been updated for decades. This was a nationwide survey conducted in Japan. Departments at hospitals were selected using stratified random sampling based on specialty and hospital size. The number of patients with ADPKD not receiving kidney replacement therapy (KRT), defined in this study as those who did not receive dialysis and did not newly undergo kidney transplantation in 2022, was estimated by taking into account sampling fractions and response rates. Data on dialysis and kidney transplantation were obtained from the Web-Based Analysis of Dialysis Data Archives (WADDA) system and the Japanese Renal Transplant Registry. Among 11,690 departments, 2,148 were selected (nephrology, n = 842; urology, n = 678; pediatrics, n = 628). The response rate was 32.9%. In 2022, the estimated number of patients with ADPKD not receiving KRT was 40,901 (95% confidence interval [CI], 29,543-52,259), corresponding to 32.7 per 100,000 population (1 in 3,055 individuals). Additionally, 12,487 patients with polycystic kidney disease (PKD) were receiving dialysis and 130 underwent kidney transplantation in 2022. After incorporating these data, the total population potentially representing ADPKD was estimated at 53,518 (42.8 per 100,000 population; 1 in 2,335 individuals). This nationwide survey provides updated epidemiological estimates of ADPKD. Although registry data on KRT may not exclusively represent ADPKD, our findings suggest a higher disease burden than previously recognized, with prevalence consistent with estimates reported in other countries.