Kidney Week

Abstract: TH-PO439

Evaluation of the Predictive Ability and Concordance of Prognostic Scores for Rapid Progression in ADPKD: A Multicenter Cohort

Session Information

• Genetic Diseases: Cystic - Therapeutic Investigations and Prognosis
  November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Genetic Diseases of the Kidneys

• 1201 Genetic Diseases of the Kidneys: Cystic

Authors

• Capelli, Irene, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Emilia-Romagna, Italy

Irene Capelli,

• Aiello, Valeria, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Emilia-Romagna, Italy

Valeria Aiello,

• Elhassan, Elhussein Aamir Elzein, Beaumont Hospital, Department of Nephrology and Transplantation, Dublin, Ireland

Elhussein Aamir Elzein Elhassan,

• Benson, Katherine A., Royal College of Surgeons in Ireland Faculty of Medicine and Health Sciences, Dublin, Ireland

Katherine A. Benson,

• Cristalli, Carlotta P., IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Emilia-Romagna, Italy

Carlotta P. Cristalli,

• Vella, Anna, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Emilia-Romagna, Italy

Anna Vella,

• Ciurli, Francesca, Universita degli Studi di Bologna, Bologna, Emilia-Romagna, Italy

Francesca Ciurli,

• Montanari, Francesca, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Emilia-Romagna, Italy

Francesca Montanari,

• La Manna, Gaetano, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Emilia-Romagna, Italy

Gaetano La Manna,

• Halbritter, Jan, Charite Universitatsmedizin Berlin, Berlin, Berlin, Germany

Jan Halbritter,

• Conlon, Peter J., Beaumont Hospital, Department of Nephrology and Transplantation, Dublin, Ireland

Peter J. Conlon,

Background

ADPKD is characterized by the progressive development of bilateral renal cysts, resulting in enlargement of the kidney volume and ESKD. ERK-NET assessed Tolvaptan indications according to 3 algorithmic criteria: total kidney volume (HtTKV) and Mayo Clinic Imaging Class (MCIC), rate of decline in eGFR, and the Predicting Renal Outcome in Polycystic Kidney Disease (PROPKD) score. These scores are alternatively used to define rapid progressor (RP) patients. The aim of this study is to evaluate and improve the concordance of sensitivity and specificity of MCIC and PROPKD predictive abilities for rapid disease progression.

Methods

Data from 3 centers (Bologna, Dublin, Berlin/Leipzig) were assessed. We defined RP with: eGFR slope ≥3 mL/min/1.73m²/ yearly over 4 years, or MCIC classes 1C-D-E, or PROPKD score (7-9). Descriptive statistics were used to summarize clinical parameters. The concordance between MCIC and PROPKD was assessed using Kappa statistics. In PKD1 missense variants, the REVEL score was obtained and treated as a continuous variable; score greater than 0.65 were considered ‘pathogenic’ and regarded as PKD1-truncating variants for PROPKD score calculation.

Results

We evaluated 298 ADPKD patients, demographic and clinical data are summarized in Tab 1. After 4 yr of follow-up, MCIC (p 0,041), HBP (p 0,031), and urological events (p<0.001) result were statistically significant on multivariate analysis (Tab 1).Assessment of RP using PROPKD and MCIC scores yielded Kappa Cohen of 0,149; 47.9% (n=143) were concordant, 49,32 % (n=148) patients identified as RP for MCIC were non- RP for PROPKD, while 2.3% (n=7) of PROPKD score considered RP using PROPKD score were considered non-RP using MCIC classes. Following the reclassification of PKD1 missense variants by REVEL score, K of Cohen improved to 0.174, and PROPKD becomes predictive of RP also at multivariate (p 0.010).

Conclusion

Concordance between scores results low (K of Cohen 0,149). PROPKD is more selective compared to the Mayo. Nevertheless, PROPKD allows the identification of some RP patients excluded from MCIC. The combined use of scoring may increase the ability to identify progressive patients. REVEL score could improve the agreement.