Myeloperoxidase-specific antineutrophil cytoplasmic antibody-associated vasculitis

Sabrina Arnold, A. Richard Kitching, Veronique Witko-Sarsat, Thorsten Wiech, Ulrich Specks, Sebastian Klapa, Sara Comdühr, Anja Stähle, Antje Müller, Peter Lamprecht*

*Corresponding author for this work

Abstract

Myeloperoxidase (MPO)-specific antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (MPO-ANCA-associated vasculitis) is one of two major ANCA-associated vasculitis variants characterised by systemic necrotising vasculitis with few or no immune deposits. MPO-ANCA-associated vasculitis predominantly affects small blood vessels and, in contrast to its counterpart proteinase 3-ANCA-associated vasculitis, is generally not associated with granulomatous inflammation. The kidneys and lungs are the most commonly affected organs. The pathogenesis of MPO-ANCA-associated vasculitis is characterised by loss of tolerance to the neutrophil enzyme MPO. This loss of tolerance leads to a chronic immunopathological response where neutrophils become both the target and effector of autoimmunity. MPO-ANCA drives neutrophil activation, leading in turn to tissue and organ damage. Clinical trials have improved the therapeutic approach to MPO-ANCA-associated vasculitis. However, there remains substantial unmet need regarding relapse frequency, toxicity of current treatment, and long-term morbidity. In this Series paper, we present the current state of research regarding pathogenesis, diagnosis, and treatment of MPO-ANCA-associated vasculitis.

Original languageEnglish
JournalThe Lancet Rheumatology
Volume6
Issue number5
Pages (from-to)e300-e313
DOIs
Publication statusPublished - 05.2024

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)

DFG Research Classification Scheme

  • 2.21-05 Immunology
  • 2.22-18 Rheumatology

Fingerprint

Dive into the research topics of 'Myeloperoxidase-specific antineutrophil cytoplasmic antibody-associated vasculitis'. Together they form a unique fingerprint.

Cite this