Immunopathogenesis of ANCA-associated vasculitis

Andreas Kronbichler, Keum Hwa Lee, Sara Denicolò, Daeun Choi, Hyojeong Lee, Donghyun Ahn, Kang Hyun Kim, Ji Han Lee, Hyungtae Kim, Minha Hwang, Sun Wook Jung, Changjun Lee, Hojune Lee, Haejune Sung, Dongkyu Lee, Jaehyuk Hwang, Sohee Kim, Injae Hwang, Do Young Kim, Hyung Jun KimGeonjae Cho, Yunryoung Cho, Dongil Kim, Minje Choi, Junhye Park, Junseong Park, Kalthoum Tizaoui, Han Li, Lee Smith, Ai Koyanagi, Louis Jacob, Philipp Gauckler, Jae Il Shin

Research output: Contribution to journalReview articlepeer-review

65 Citations (Scopus)


Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disorder which affects small-and, to a lesser degree, medium-sized vessels. ANCA-associated vasculitis encompasses three disease phenotypes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). This classification is largely based on clinical presentations and has several limitations. Recent research provided evidence that genetic background, risk of relapse, prognosis, and co-morbidities are more closely related to the ANCA serotype, proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA, compared to the disease phenotypes GPA or MPA. This finding has been extended to the investigation of biomarkers predicting disease activity, which again more closely relate to the ANCA serotype. Discoveries related to the immunopathogenesis translated into clinical practice as targeted therapies are on the rise. This review will summarize the current understanding of the immunopathogenesis of ANCA-associated vasculitis and the interplay between ANCA serotype and proposed disease biomarkers and illustrate how the extending knowledge of the immunopathogenesis will likely translate into development of a personalized medicine approach in the management of ANCA-associated vasculitis.

Original languageEnglish
Article number7319
Pages (from-to)1-27
Number of pages27
JournalInternational journal of molecular sciences
Issue number19
Publication statusPublished - 2020 Oct 1

Bibliographical note

Publisher Copyright:
© 2020 by the authors. Licensee MDPI, Basel, Switzerland.

All Science Journal Classification (ASJC) codes

  • Catalysis
  • Molecular Biology
  • Spectroscopy
  • Computer Science Applications
  • Physical and Theoretical Chemistry
  • Organic Chemistry
  • Inorganic Chemistry


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