Cellular immune regulation in the pathogenesis of ANCA-associated vasculitides

Anouk von Borstel, Jan Stephan Sanders, Abraham Rutgers, Coen A. Stegeman, Peter Heeringa, Wayel H. Abdulahad

Research output: Contribution to journalReview ArticleResearchpeer-review

23 Citations (Scopus)


Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are systemic autoimmune diseases characterized by necrotizing inflammation of small- to medium-sized blood vessels, affecting primarily the lungs and kidneys. Both animal and human studies show that the balance between inflammatory- and regulatory T- and B cells determines the AAV disease pathogenesis. Recent evidence shows malfunctioning of the regulatory lymphocyte compartment in AAV. In this review we summarize the immune regulatory properties of both T- and B cells in patients with AAV and discuss how aberrations herein might contribute to the disease pathogenesis.

Original languageEnglish
Pages (from-to)413-421
Number of pages9
JournalAutoimmunity Reviews
Issue number4
Publication statusPublished - 1 Apr 2018
Externally publishedYes


  • ANCA
  • B regulatory cells
  • T regulatory cells
  • Vasculitides

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