B cell phenotype in pediatric idiopathic nephrotic syndrome

Pediatr Nephrol. 2019 Jan;34(1):177-181. doi: 10.1007/s00467-018-4095-z. Epub 2018 Sep 28.

Abstract

Background: A pathogenic role of B cells in non-genetic nephrotic syndrome has been suggested by the efficacy of rituximab, a B cell depleting antibody, in maintaining a prolonged remission. However, little information is available on B cell homeostasis in nephrotic syndrome patients.

Methods: We retrospectively analyzed by flow cytometry the distribution of different B cell subpopulations in 107 steroid-sensitive and in 6 genetic steroid-resistant nephrotic syndrome pediatric patients, compared with age- and sex-matched controls.

Results: Fifty-one steroid-sensitive patients at disease onset, before starting immunosuppression, presented significantly increased levels of total, transitional, memory, and switched memory B cells compared to controls. Oral immunosuppression strongly affected transitional and mature B cell levels in 27 patients in relapse and also in 29 patients in remission, whereas memory B cells were significantly higher compared to controls during relapse, despite the immunosuppressive treatment, and were normalized only in patients in remission. Children with genetic forms of steroid-resistant nephrotic syndrome presented no differences in B cell profile from controls.

Conclusions: Our study indicates that memory B cells, more than other B cell subsets, are increased and appear to be pathogenically relevant in steroid-sensitive nephrotic syndrome pediatric patients.

Keywords: B cells; Children; Immunosuppressive treatment; Nephrotic syndrome.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • B-Lymphocyte Subsets / immunology*
  • Cell Separation
  • Child
  • Child, Preschool
  • Drug Resistance
  • Flow Cytometry
  • Glucocorticoids / pharmacology*
  • Glucocorticoids / therapeutic use
  • Healthy Volunteers
  • Humans
  • Immunologic Memory*
  • Infant
  • Male
  • Nephrotic Syndrome / blood
  • Nephrotic Syndrome / drug therapy
  • Nephrotic Syndrome / immunology*
  • Retrospective Studies
  • Young Adult

Substances

  • Glucocorticoids