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Remission of proteinuria in multidrug-resistant idiopathic nephrotic syndrome following immunoglobulin

Elodie Nattes1, Danièle Karaa1, Laurène Dehoux1

  • 1Pediatric Nephrology Unit, Hôpital Robert Debré, APHP, Sorbonne Paris Cité University, Paris, France.

Acta Paediatrica (Oslo, Norway : 1992)
|September 20, 2018
PubMed

Insights

Multidrug-resistant nephrotic syndrome in children can be treated with immunoglobulin immunoadsorption (IgIA) and B-cell depletion, achieving remission in most patients. However, sustained remission often requires additional immunosuppression.

Area of Science:

  • Pediatric Nephrology
  • Immunology

Background:

  • Idiopathic nephrotic syndrome (INS) resistant to standard treatments like prednisone and calcineurin inhibitors can progress to end-stage renal disease.
  • Multidrug-resistant INS poses a significant therapeutic challenge in pediatric patients.

Purpose of the Study:

  • To evaluate the efficacy of a treatment combining immunoglobulin immunoadsorption (IgIA), intravenous immunoglobulins, and B-cell depletion for multidrug-resistant nephrotic syndrome in children.
  • To report the outcomes and remission rates in this patient cohort.

Main Methods:

  • Patients underwent ten sessions of IgIA and intravenous immunoglobulins over two weeks.
  • Rituximab was administered for patients achieving remission.
  • Treatment outcomes, including proteinuria remission and relapse rates, were monitored.

Main Results:

  • Remission of proteinuria was achieved in 9 out of 14 patients after the initial phase, with 2 additional patients responding after extended treatment.
  • Remission was stable in 3 patients and maintained with additional IgIA and immunosuppression in 6 patients.
  • Two patients relapsed after IgIA withdrawal, and 3 patients (including 2 with genetic podocytopathy) did not respond to treatment.

Conclusions:

  • Immunoglobulin immunoadsorption (IgIA) can induce remission in children with multidrug-resistant nephrotic syndrome, potentially preventing progression to end-stage renal disease.
  • Achieving and maintaining remission often necessitates significant additional immunosuppressive therapy.
Abstract

Keywords:
AntibodyB cellIntravenous immunoglobulin GProteinuriaRituximab

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