Evidence for a pathogenic role of extrafollicular, IL-10-producing CCR6+B helper T cells in systemic lupus erythematosus

Proc Natl Acad Sci U S A. 2020 Mar 31;117(13):7305-7316. doi: 10.1073/pnas.1917834117. Epub 2020 Mar 17.

Abstract

Interleukin 10 (IL-10) is an antiinflammatory cytokine, but also promotes B cell responses and plays a pathogenic role in systemic lupus erythematosus (SLE). CD4+CCR6+IL-7R+T cells from human tonsils produced IL-10 following stimulation by naïve B cells, which promoted B cell immunoglobulin G (IgG) production. These tonsillar CCR6+B helper T cells were phenotypically distinct from follicular helper T (TFH) cells and lacked BCL6 expression. In peripheral blood, a CCR6+T cell population with similar characteristics was identified, which lacked Th17- and TFH-associated gene signatures and differentiation-associated surface markers. CD4+CCR6+T cells expressing IL-10, but not IL-17, were also detectable in the spleens of cytokine reporter mice. They provided help for IgG production in vivo, and expanded systemically in pristane-induced lupus-like disease. In SLE patients, CD4+CCR6+IL-7R+T cells were associated with the presence of pathogenic anti-dsDNA (double-stranded DNA) antibodies, and provided spontaneous help for autoantibody production ex vivo. Strikingly, IL-10-producing CCR6+T cells were highly abundant in lymph nodes of SLE patients, and colocalized with B cells at the margins of follicles. In conclusion, we identified a previously uncharacterized population of extrafollicular B helper T cells, which produced IL-10 and could play a prominent pathogenic role in SLE.

Keywords: B cell help; interleukin-10; lupus.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Animals
  • Antibody Formation
  • B-Lymphocytes / immunology*
  • Child
  • Cytokines / immunology
  • Humans
  • Interleukin-10 / biosynthesis
  • Interleukin-10 / immunology*
  • Interleukin-17 / metabolism
  • Lupus Erythematosus, Systemic / immunology*
  • Lupus Erythematosus, Systemic / metabolism
  • Mice
  • Mice, Inbred C57BL
  • Palatine Tonsil / cytology
  • Palatine Tonsil / immunology
  • Receptors, CCR6 / biosynthesis
  • Receptors, CCR6 / immunology*
  • T-Lymphocytes, Helper-Inducer / immunology*
  • Th17 Cells / immunology

Substances

  • CCR6 protein, human
  • CCR6 protein, mouse
  • Cytokines
  • IL10 protein, human
  • IL10 protein, mouse
  • Interleukin-17
  • Receptors, CCR6
  • Interleukin-10