The extrafollicular B cell response is a hallmark of childhood idiopathic nephrotic syndrome

Tho-Alfakar Al-Aubodah, Lamine Aoudjit, Giuseppe Pascale, Maneka A. Perinpanayagam, David Langlais, Martin Bitzan, Susan M. Samuel, Ciriaco A. Piccirillo () and Tomoko Takano ()
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Tho-Alfakar Al-Aubodah: McGill University
Lamine Aoudjit: Research Institute of the McGill University Health Centre
Giuseppe Pascale: McGill University
Maneka A. Perinpanayagam: University of Calgary
David Langlais: McGill University
Martin Bitzan: McGill University
Susan M. Samuel: University of Calgary
Ciriaco A. Piccirillo: McGill University
Tomoko Takano: Research Institute of the McGill University Health Centre

Nature Communications, 2023, vol. 14, issue 1, 1-15

Abstract: Abstract The efficacy of the B cell-targeting drug rituximab (RTX) in childhood idiopathic nephrotic syndrome (INS) suggests that B cells may be implicated in disease pathogenesis. However, B cell characterization in children with INS remains limited. Here, using single-cell RNA sequencing, we demonstrate that a B cell transcriptional program poised for effector functions represents the major immune perturbation in blood samples from children with active INS. This transcriptional profile was associated with an extrafollicular B cell response marked by the expansion of atypical B cells (atBCs), marginal zone-like B cells, and antibody-secreting cells (ASCs). Flow cytometry of blood from 13 children with active INS and 24 healthy donors confirmed the presence of an extrafollicular B cell response denoted by the expansion of proliferating RTX-sensitive extrafollicular (CXCR5–) CD21low T-bet+ CD11c+ atBCs and short-lived T-bet+ ASCs in INS. Together, our study provides evidence for an extrafollicular origin for humoral immunity in active INS.

Date: 2023
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DOI: 10.1038/s41467-023-43504-8

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