Renal transplant outcomes in patients with autosomal dominant tubulointerstitial kidney disease

Clin Transplant. 2020 Feb;34(2):e13783. doi: 10.1111/ctr.13783. Epub 2020 Feb 5.


Introduction: Autosomal dominant tubulointerstitial kidney disease (ADTKD) is a rare genetic cause of chronic kidney disease (CKD) and end-stage renal disease (ESRD). We aimed to compare renal transplant outcomes in people with ESRD due to ADTKD to those with other causes of renal failure.

Methods: Patients with clinical characteristics consistent with ADTKD by the criteria outlined in the 2015 KDIGO consensus were included. We compared ADTKD transplant outcomes with those of 4633 non-ADTKD renal transplant recipients.

Results: We included 31 patients who met diagnostic criteria for ADTKD in this analysis, 23 of whom had an identified mutation (28 were categorized as definite-ADTKD and 3 as suspected ADTKD). Five patients received a second transplant during follow-up. In total, 36 grafts were included. We did not identify significant differences between groups in terms of graft or patient survival after transplantation. Twenty-five transplant biopsies were performed during follow-up, and none of these showed signs of recurrent ADTKD post-transplant.

Conclusion: In patients with ESRD due to ADTKD, we demonstrate that transplant outcomes are comparable with the general transplant population. There is no evidence that ADTKD can recur after transplantation.

Keywords: ADTKD; genetics; graft survival; kidney disease; rare renal disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Graft Survival
  • Humans
  • Kidney Failure, Chronic* / surgery
  • Kidney Transplantation*
  • Mutation
  • Polycystic Kidney, Autosomal Dominant*
  • Uromodulin / genetics


  • Uromodulin