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Abstract: SA-PO0550

Factors Associated with Loss of Kidney Function in Patients with PKD: A Nationwide Real-World Data Analysis

Session Information

Category: Genetic Diseases of the Kidneys

  • 1201 Genetic Diseases of the Kidneys: Monogenic Kidney Diseases

Authors

  • Hoshino, Junichi, Tokyo Joshi Ika Daigaku, Shinjuku, Tokyo, Japan
  • Kataoka, Hiroshi, Tokyo Joshi Ika Daigaku, Shinjuku, Tokyo, Japan
  • Nishio, Saori, Hokkaido Daigaku, Sapporo, Hokkaido Prefecture, Japan
  • Hiromura, Keiju, Gunma Daigaku, Maebashi, Gunma Prefecture, Japan
  • Isaka, Yoshitaka, Osaka Daigaku, Suita, Osaka Prefecture, Japan
  • Muto, Satoru, Juntendo Daigaku, Bunkyo, Tokyo, Japan

Group or Team Name

  • The PKD Working Group, Investigate Research on Refractory Kidney Disorders in Japan.
Background

Autosomal dominant polycystic kidney disease (ADPKD) is associated with a high risk of developing end-stage renal disease (ESRD). While genetic factors are fixed, other modifiable factors prevent disease progression. This study examined factors associated with ESRD in ADPKD patients.

Methods

Using Japanese national registry data from 2015–2021, we analyzed the decrease in the estimated glomerular filtration rate (eGFR) and related factors in 3,964 ADPKD patients who met the intractable disease criteria in Japan and were followed for more than three years (mean age, 50 years; 52% males). The primary outcome was a 30% decrease in the eGFR over 3 years, and factors associated with the decrease in the eGFR were analyzed. To identify risk factors, a Cox proportional hazards model and classification and regression tree (CART) analysis were performed.

Results

The mean eGFR decline was -3.22±2.98 mL/min/1.73 m2/year, with a greater decline in patients with greater kidney volume and advanced chronic kidney disease (CKD) stages. In the multivariate Cox analyses, CKD stages G3a, G3b, and G4 had hazard ratios (HRs) for poor renal outcomes of 1.89 (1.40–2.56), 3.84 (2.93–5.05), and 7.24 (5.52–9.51), respectively, and proteinuria categories A2 and A3 had HRs of 1.66 (1.43–1.93) and 2.02 (1.71–2.40), respectively. Conversely, protective factors included older age (HR, 0.82 [0.78–0.88]), male sex (HR, 0.85 [0.75–0.97]), and tolvaptan use (HR, 0.79 [0.67–0.93]). The CART analysis suggested that CKD G4 patients, particularly those aged <60 years, those aged 60-68 years with proteinuria, and CKD G3b patients aged <54 years with proteinuria had over 50% risks of ESRD.

Conclusion

This study focused on PKD patients already identified as being at risk for ESRD (patients with large TKVs or classified in the red zone of the KDIGO heat map). Within this high-risk group, CKD G4 patients aged <60 years, those aged 60-68 years with proteinuria, and CKD G3b patients aged <54 years with proteinuria, presented exceptionally high ESRD risk. These findings suggest that these individuals require intensive care, including early lifestyle interventions, dietary guidance, and pharmacological treatment, to delay disease progression.

Funding

  • Government Support – Non-U.S.

Digital Object Identifier (DOI)