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Kidney Week

Abstract: TH-PO436

Prognosis of Polycystic Kidney Disease: FinnGen Study

Session Information

Category: Genetic Diseases of the Kidneys

  • 1201 Genetic Diseases of the Kidneys: Cystic

Authors

  • Visser, Tomas Juhani, Helsingin yliopisto, Helsinki, Uusimaa, Finland
  • Hellman, Tapio, TYKS Turu yliopistollinen keskussairaala, Turku, Varsinais-Suomi, Finland
  • Kauko, Anni, Turun yliopisto, Turku, Varsinais-Suomi, Finland
  • Simons, Matias, University Hospital of Heidelberg, Heidelberg, Germany
  • Jahnukainen, Timo, HUS-yhtyma, Helsinki, Uusimaa, Finland
  • Helve, Jaakko, Finnish Registry of Kidney Diseases, Helsinki, Uusimaa, Finland
  • Finne, Patrik, HUS-yhtyma, Helsinki, Uusimaa, Finland
  • Gordin, Daniel, HUS-yhtyma, Helsinki, Uusimaa, Finland
  • Niiranen, Teemu, Turun yliopisto, Turku, Varsinais-Suomi, Finland
Background

The extra-renal complication risk and progression rate of autosomal dominant polycystic kidney disease (PKD) are incompletely understood. We assessed the clinical outcomes of PKD in the FinnGen study, which covers nearly 10% of the Finnish population (N=473681).

Methods

PKD patients and clinical outcomes were identified using ICD diagnosis codes. The outcomes of interest were death, start of dialysis or transplantation (RRT), major adverse cardiovascular events (MACE), subarachnoid hemorrhage (SAH) or cerebral aneurysms, valvular heart disease (VHD, defined as valvular regurgitation), or diverticular disease. In a smaller sample with data available for estimated glomerular filtration rate (eGFR), we compared the risk of adverse outcomes and kidney function decline in PKD patients and controls (diabetes mellitus [DM] patients) using multivariable-adjusted Cox regression and repeated linear mixed models. The cases and controls were matched with a ratio of 1:4 by age, sex and eGFR.

Results

The study sample included 674 PKD patients. The incidence of clinical outcomes in these patients is reported in Figure 1. The risk of adverse outcomes in PKD patients (N=173) compared to DM patients (N=692) is reported in Figure 2. The risk of death, RRT, SAH or cerebral aneurysms, and diverticular disease was greater in PKD patients compared to DM patients (p<0.02 for all). The eGFR decreased more rapidly in PKD patients compared to DM patients (-2.31 [95% CI, -2.41 to -2.22] vs -1.62 [95% CI, -1.67 to -1.56] ml/min/1.73 m2 per year, p<1x10-16).

Conclusion

In this large register study, PKD patients demonstrated a greater risk for most adverse outcomes and a more rapid kidney function decline compared to DM patients.