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Clinical Trial ARTICLE Provisionally accepted The full-text will be published soon. Notify me

Front. Pediatr. | doi: 10.3389/fped.2019.00454

Dextran-sulfate plasma adsorption lipoprotein apheresis in drug resistant primary focal segmental glomerulosclerosis patients: Results from a prospective, multicenter, single-arm intervention study

 Rupesh Raina1, 2*,  Vinod Krishnappa2,  Cheryl Sanchez-Kazi3, Alejandro Quiroga4,  Katherine Twombley5, Robert Mathias6, Megan Lo7, Shefali Mahesh1 and Joshua Zaritsky8
  • 1Akron Children's Hospital, United States
  • 2Cleveland Clinic Akron General, United States
  • 3Loma Linda University Children's Hospital, United States
  • 4Helen DeVos Children's Hospital, United States
  • 5Medical University of South Carolina, United States
  • 6Nemours Children's Hospital, United States
  • 7Children's Hospital of Richmond at VCU, United States
  • 8Alfred I. duPont Hospital for Children, United States

Background: Focal segmental glomerulosclerosis (FSGS) causes end stage renal disease (ESRD) in significant proportion of patients worldwide. Primary FSGS carries poor prognosis and management of FSGS patients, refractory to standard treatments or resistant to steroids, remains a major challenge. Lipoprotein apheresis is a therapeutic approach for drug resistant primary FSGS and post-renal transplant primary FSGS recurrence.
Objectives: To examine the safety and probable benefit at 1, 3, 6, 12 and 24-months following completion of apheresis treatment using Liposorber® LA-15 system in patients with nephrotic syndrome (NS), due to refractory primary FSGS or primary FSGS associated NS, in post renal transplant children.
Material and methods: Prospective, multicenter, single-arm intervention study using Liposorber® LA-15 system. Patients ≤21 years old with drug resistant or drug intolerant NS secondary to primary FSGS with glomerular filtration rate (GFR) ≥60 ml/min/1.73m2 or post renal transplant patients ≤21 years old with primary FSGS associated NS were included in the study. Each patient had 12 dextran-sulfate plasma adsorption lipoprotein apheresis sessions over a period of 9 weeks. All patients were followed up at 1, 3, 6, 12 and 24-months following completion of treatment.
Results: Of 17 patients enrolled, six were excluded from the outcome analysis (protocol deviations). Of the remaining 11 patients, all but one have completed apheresis treatments. Three patients were lost to follow-up immediately after completion of apheresis and excluded from outcome analysis. At one-month follow-up, 1 of 7 patients (14.3%) attained partial remission of NS while 2 of 4 subjects (50%) and 2 of 3 subjects (66.7%) had partial/complete remission at 3- and 6-months follow-up, respectively. One of two patients followed up for 12 months had complete remission and one patient had partial remission of NS after 24 months. Improved or stable eGFR was noted in all patients over the follow-up period.
Conclusion: The results of our multicenter study showed improvement in the response rates to steroid or immunosuppressive therapy and induced complete or partial remission of proteinuria in some of the patients with drug resistant primary FSGS. The main limitation of our study is the small number of subjects and high dropout rate.

Keywords: Focal segmental glomerulosclerosis (FSGS), Nephrotic Syndrome, iposorber, Proteinuria, Lipoprotein apheresis (LA)

Received: 13 May 2019; Accepted: 18 Oct 2019.

Copyright: © 2019 Raina, Krishnappa, Sanchez-Kazi, Quiroga, Twombley, Mathias, Lo, Mahesh and Zaritsky. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Mx. Rupesh Raina, Akron Children's Hospital, Akron, United States, rraina@akronchildrens.org