Mader G, Purser M, Mladsi D, Sanon M, Oberdhan D, Watnick T, Seliger S. Modeling disease progression in patients at risk of rapid progression in Autosomal Dominant Polycystic Kidney Disease (ADPKD): using the mayo classification prediction equation. Poster presented at the 2019 National Kidney Foundation Conference; May 9, 2019. Boston, MA.

INTRODUCTION: ADPKD patients progress at variable rates, and the Mayo classification system has been identified as a robust tool to predict progression in rapidly progressing ADPKD (subclasses 1C, 1D, and 1E). A de novo cohort model was developed to predict long-term outcomes of patients at risk for rapid progression.

METHODS: The model predicts eGFR decline among patients with ADPKD at risk for rapid progression based on the Mayo classification system, via the “Irazabal equation”, which estimates eGFR decline as a function of an individual’s current eGFR and age-specific height-adjusted total kidney volume. The model combines patient-level progression estimates to simulate a cohort with rapidly progressing ADPKD progressing at different rates. The model base-case population represents rapid progressors from the TEMPO 3:4 clinical trial beginning in CKD stages 1, 2, and 3 across ADPKD subclasses 1C, 1D, and 1E. Using this model, we estimated lifetime eGFR decline through progressive CKD stages and into ESRD, dialysis, and transplantation.

RESULTS: For patients beginning in CKD 1, 2, and 3, the model estimates time to ESRD to be 18.4 years in CKD 1 (20.1 years for 1C, 18.2 years 1D, and 15.2 years 1E), 12.5 years in CKD 2 (14.2 years 1C, 12.0 years 1D, and 9.2 years 1E), and 7.6 years in CKD 3 (9.5 years 1C, 7.8 years 1D, and 5.2 years 1E).

CONCLUSION: Among patients with higher-risk Mayo ADPKD classes, a lifetime disease progression model predicts that patients spend less time in the earlier stages of CKD and, consequently, more time in ESRD. Results highlight the importance of accounting for rapid progressors in estimates of eGFR decline and the need for treatments to delay time to ESRD for patients with rapidly progressing disease.

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