Renal replacement therapy for autosomal dominant polycystic kidney disease (ADPKD) in Europe: prevalence and survival--an analysis of data from the ERA-EDTA Registry

Abstract

Background: Autosomal dominant polycystic kidney disease (ADPKD) is the fourth most common renal disease requiring renal replacement therapy (RRT). Still, there are few epidemiological data on the prevalence of, and survival on RRT for ADPKD.

Methods: This study used data from the ERA-EDTA Registry on RRT prevalence and survival on RRT in 12 European countries with 208 million inhabitants. We studied four 5-year periods (1991-2010). Survival analysis was performed by the Kaplan-Meier method and by Cox proportional hazards regression.

Results: From the first to the last study period, the prevalence of RRT for ADPKD increased from 56.8 to 91.1 per million population (pmp). The percentage of prevalent RRT patients with ADPKD remained fairly stable at 9.8%. Two-year survival of ADPKD patients on RRT (adjusted for age, sex and country) increased significantly from 89.0 to 92.8%, and was higher than for non-ADPKD subjects. Improved survival was noted for all RRT modalities: haemodialysis [adjusted hazard ratio for mortality during the last versus first time period 0.75 (95% confidence interval 0.61-0.91), peritoneal dialysis 0.55 (0.38-0.80) and transplantation 0.52 (0.32-0.74)]. Cardiovascular mortality as a proportion of total mortality on RRT decreased more in ADPKD patients (from 53 to 29%), than in non-ADPKD patients (from 44 to 35%). Of note, the incidence rate of RRT for ADPKD remained relatively stable at 7.6 versus 8.3 pmp from the first to the last study period, which will be discussed in detail in a separate study.

Conclusions: In ADPKD patients on RRT, survival has improved markedly, especially due to a decrease in cardiovascular mortality. This has led to a considerable increase in the number of ADPKD patients being treated with RRT.

Keywords: ADPKD; epidemiology; prevalence; renal replacement therapy; survival.

Figure 1

Prevalence of renal replacement therapy (RRT) for patients with ADPKD. Data are the average of the period 2006 through 2010, expressed per million population (pmp), and adjusted for age and sex distribution of the 2005 EU27 population.

Figure 2

Prevalence of renal replacement therapy for patients with ADPKD versus non-ADPKD kidney diseases. Data are the average of the period 2006 through 2010, expressed per million population, and adjusted for age and sex to the distribution of the 2005 EU27 population. The size of marker denotes the size of the general population under study. Abbreviations: AT, Austria; BE, Belgium; DK, Denmark; ES, Spain; FI, Finland; FR, France; GR, Greece; IT, Italy, Calabria; NL, The Netherlands; RO, Romania; SE, Sweden; UK, United Kingdom.

Figure 3

Trends in prevalence of ADPKD patients on renal replacement therapy as percentage of the total population on renal replacement therapy. Abbreviations: AT, Austria; BE, Belgium; DK, Denmark; ES, Spain; FI, Finland; FR, France; GR, Greece; IT, Italy, Calabria; NL, The Netherlands; RO, Romania; SE, Sweden; UK, United Kingdom. * ; Coverage increasing over time, see for details Supplementary Table 1.⁺ ; All countries with complete follow-up coverage.

Figure 4

Patients on hemodialysis (upper panel), peritoneal dialysis (middle panel) and living with a renal transplant (lower panel) as percentage of the total population on renal replacement therapy with ADPKD versus non-ADPKD. Data are the average of the period 2006 through 2010, and adjusted for age and sex to the distribution of the EU27 population in 2005. The size of marker denotes the size of the general population under study. Abbreviations AT, Austria; BE, Belgium; DK, Denmark; ES, Spain; FI, Finland; FR, France; GR, Greece; IT, Italy, Calabria; NL, The Netherlands; RO, Romania; SE, Sweden; UK, United Kingdom.

Figure 5

Trends in two-year patient survival in ADPKD versus non-ADPKD patients aged 60-65 years at onset of renal replacement therapy, overall and per specific treatment modality. Adjusted for age at start RRT, sex and primary renal disease (diabetes, hypertension, glomerulonephritis and other). Survival probabilities are standardised according to the following fixed values: age=60, males=60%, diabetes=20%, hypertension=17% and glomerulonephritis=15%). Abbreviations: RRT, renal replacement therapy; HD, hemodialysis; PD, peritoneal dialysis; RTR, renal transplant recipients.

Figure 6

Trends in causes of death in patients on renal replacement therapy for ADPKD and non-ADPKD. Adjusted for to average age and gender distribution of all patients starting RRT between 1991 and 2010. Abbreviation: CV, cardiovascular.