Incidence and Outcomes of Kidney Replacement Therapy for Kidney Failure Due to Inherited Tubular Disorders in Europe: Findings from the ERA Registry

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Background: Inherited tubular disorders represent a broad subgroup of genetic kidney diseases, with approximately 13% progressing to kidney failure. In 2015 the ERA Registry began collecting data on primary renal disease (PRDs) for all specific diseases. We aimed to examine the incidence of patients starting KRT due kidney failure caused by inherited tubular disorders between 2015 and 2022, overall, and by age and sex. Then we aimed to compare their survival with that of patients with other PRDs (excluding diabetes). Methods: Patients with inherited tubular disorders were identified using the most recent 2012 PRD codes that were collected since 2015. The comparators consisted of KRT patients with other PRDs than inherited tubular disorders, who were matched using age, sex and year of KRT initiation. The Kaplan-Meier method was used for patient survival analyses. Results: In total, 194 patients started KRT due to inherited tubular disorders (figure 1), the majority of them were men (60%) and younger than 65 (81%). The most common condition was primary hyperoxaluria. The overall incidence of KRT for kidney failure due to inherited tubular disorders between 2015-2022 was 0.32 per million people (pmp), accounting for only 0.24% of all incident KRT cases. After 5 years, the survival of patients with inherited tubular disorders was similar to that of the matched comparators: 76.3% (95% CI: 67.2-83.2) and 74.9% (95% CI: 70.5-78.7), respectively (p = 0.630). Conclusion: The update of the ERA PRD codes made it possible to study the epidemiology of patients on KRT caused by inherited tubular disorders, yet they are rarely registered as PRD. After five years, their survival was similar to that of other KRT patients with the same age and sex.Figure 1 Distribution of specific PRDs for patients who started KRT for inherited tubulopathies between 2015 and 2022 , overall (A), by age (B), and sex (C).

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