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Abstract: PO1549

Kidney Outcomes in Biopsy-Proven Thrombotic Microangiopathy with Eculizumab Therapy

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Sohail, Mohammad Ahsan, Cleveland Clinic, Cleveland, Ohio, United States
  • Sedor, John R., Cleveland Clinic Glickman Urological and Kidney Institute, Cleveland, Ohio, United States
  • Arrigain, Susana, Cleveland Clinic Glickman Urological and Kidney Institute, Cleveland, Ohio, United States
  • Schold, Jesse D., Cleveland Clinic Glickman Urological and Kidney Institute, Cleveland, Ohio, United States
  • Herlitz, Leal C., Cleveland Clinic Glickman Urological and Kidney Institute, Cleveland, Ohio, United States
  • Mehdi, Ali, Cleveland Clinic Glickman Urological and Kidney Institute, Cleveland, Ohio, United States
Background

There are limited long-term data on kidney outcomes in eculizumab-treated patients with biopsy-proven thrombotic microangiopathy (TMA). We report our experience of using eculizumab in patients with primary (genetic/antibody mediated) TMA, secondary TMA syndromes associated with antiphospholipid syndrome (APS) or systemic lupus erythematosus (SLE), as well as TMA of undetermined etiology.

Methods

We reviewed adult patients with kidney biopsy-proven TMA treated with eculizumab between 2014-2019. Transplant recipients, pregnant patients and those with scleroderma, shiga-toxin related hemolytic uremic syndrome or thrombotic thrombocytopenic purpura were excluded. Kidney response to eculizumab at 26 weeks for patients not on kidney replacement therapy (KRT) and for those requiring KRT at the time of or within 1 week of initiation of eculizumab, was defined as an increase in eGFR of 15 ml/min/1.73m2 and liberation from KRT respectively. Death within 26 weeks was considered lack of response.

Results

We collected data on 16 patients (primary TMA [n=3]; secondary TMA including SLE [n=3] and APS [n=4]; TMA of undetermined etiology [n=6]). The median time from biopsy diagnosis to treatment initiation was 3.0 days (IQR:-1.0,10.0) and the median duration of therapy was 303 days (IQR:160,604). 13 patients (81%) required KRT at the time of initiation of eculizumab. 4 patients died during follow up, 2 of whom died within 26 weeks. 6 (37.5%) patients exhibited kidney response to eculizumab, 5 of whom required KRT when therapy was started. 2 of 3 patients who did not need KRT initially eventually progressed to end-stage kidney disease after 1 and 5.5 years from treatment initiation. 3/6 patients (50%) and 3/8 patients (37.5%) with mild and moderate interstitial fibrosis and tubular atrophy (IFTA) on biopsy respectively responded to therapy, whilst those with severe IFTA (n=2) showed no response.

Conclusion

Although eculizumab use has been expanding rapidly for primary and secondary TMA syndromes, our data depicts a suboptimal kidney response, which appears independent of the need for KRT at treatment initiation. Severity of IFTA may be a predictor of kidney response to eculizumab. We suggest that more data is needed on long-term kidney outcomes with eculizumab across TMA syndromes before universally adopting this expensive therapeutic strategy.