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Kidney Week

Abstract: FR-OR088

National Observational Study Monitoring the Restrictive Regimen of Eculizumab Therapy in aHUS in the Netherlands

Session Information

  • Lupus and Then Some
    November 08, 2019 | Location: Ballroom C, Walter E. Washington Convention Center
    Abstract Time: 05:54 PM - 06:06 PM

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Bouwmeester, Romy N., Radboudumc, Nijmegen, Netherlands
  • Duineveld, Caroline, Radboudumc, Nijmegen, Netherlands
  • Wijnsma, Kioa L., Radboudumc, Nijmegen, Netherlands
  • Wetzels, Jack F., Radboud University Medical Center, Nijmegen, Netherlands
  • Van De Kar, Nicole, UMC St. Radboud Nymegen, Nijmegen, Netherlands

Group or Team Name

  • CUREiHUS Study Group
Background

Nowadays eculizumab is the cornerstone of treatment in atypical hemolytic uremic syndrome (aHUS). The optimal treatment strategy in aHUS is still unknown. In October 2016, a Dutch guideline promoting a restrictive eculizumab regimen in aHUS, was implemented. Here we report the preliminary results of the CUREiHUS study, monitoring this guideline.

Methods

The data of pediatric and adult Dutch aHUS patients included in CUREiHUS study from 1-10-2016 till April 2019 were evaluated. Patients were divided in two groups; a historical cohort containing aHUS patients already on eculizumab treatment before October 2016 (n=13) and aHUS cohort who started with eculizumab after October 2016 (n=24).

Results

In the historical cohort eculizumab could be withdrawn in 5 of the 13 aHUS patients (median duration treatment 6,6 months). Four patients are treated with interval elongation ranging from 3 to 6 weeks). Three patients are on two-weekly interval dosage. In two of the 24 patients who started eculizumab after October 2016 aHUS was diagnosed <3 months and are receiving the standard two-weekly regimen according to the Dutch guideline. In 22 of these 24 aHUS patients the follow up is > 3 months. In 13 patients the eculizumab therapy could be withdrawn. The median duration of treatment was 3,2 months (range 0,3-10 months). The median follow-up after the last gift of eculizumab was 17 months (range 1-35 months). Tapering of eculizumab was seen in 6 patients (interval 3-6 weeks). Additional three patients are treated with the standard two weekly regimen treatment. Relapse of HUS occurred in 14 patients and were treated immediately with eculizumab. In total 8/34 (24%) are at the moment treated with eculizumab biweekly. Eculizumab therapy is withdrawn in 38% (5/13) and 62% (13/21) in patients with onset of aHUS before and after implementation of the guideline, respectively.

Conclusion

The majority of aHUS patients do not need eculizumab biweekly and can be safely switched to an extended interval or withdrawal of therapy. Continuous monitoring and further determination of contributing or predictive factors to relapse(s) are necessary.