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

doi:10.1016/j.autrev.2017.12.002

Cellular immune regulation in the pathogenesis of ANCA-associated vasculitides

Autoimmun Rev. 2018 Apr;17(4):413-421. doi: 10.1016/j.autrev.2017.12.002. Epub 2018 Feb 9.

Authors

Anouk von Borstel¹, Jan Stephan Sanders², Abraham Rutgers³, Coen A Stegeman², Peter Heeringa⁴, Wayel H Abdulahad⁵

Affiliations

¹ Department of Internal Medicine, Division of Nephrology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen 9713 GZ, The Netherlands. Electronic address: [email protected].
² Department of Internal Medicine, Division of Nephrology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen 9713 GZ, The Netherlands.
³ Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen 9713 GZ, The Netherlands.
⁴ Department of Pathology and Medical Biology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen 9713 GZ, The Netherlands.
⁵ Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen 9713 GZ, The Netherlands; Department of Pathology and Medical Biology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen 9713 GZ, The Netherlands.

PMID: 29428808
DOI: 10.1016/j.autrev.2017.12.002

Abstract

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.

Keywords: ANCA; B regulatory cells; T regulatory cells; Vasculitides.

Publication types

Review

MeSH terms

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / immunology*
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / pathology
Humans
Immunity, Cellular / immunology*