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Almeida et al. Vessel Plus 2021;5:44 Vessel Plus
DOI: 10.20517/2574-1209.2021.66

Review Open Access

Pathogenesis and biomarkers in ANCA-associated

vasculitis

1,#
1,#
Sofia Almeida , Pedro Maia Neves , Vital Da Silva Domingues 1,2,#
1
Serviço de Medicina, Centro Hospitalar e Universitário do Porto, Porto 4099-001, Portugal.
2
Inflammation and Lymphocyte Physiology Labs, Gulbenkian Institute of Science, Oeiras 270-156, Portugal.
#
Authors contributed equally.
Correspondence to: Dr. Vital Da Silva Domingues, Serviço de Medicina, Centro Hospitalar e Universitário do Porto, Largo Prof.
Abel Salazar, Porto 4099-001, Portugal. E-mail: vdomingues@igc.gulbenkian.pt
How to cite this article: Almeida S, Neves PM, Da Silva Domingues V. Pathogenesis and biomarkers in ANCA-associated
vasculitis. Vessel Plus 2021;5:44. https://dx.doi.org/10.20517/2574-1209.2021.66
Received: 18 Apr 2021 First Decision: 18 May 2021 Revised: 20 Jun 2021 Accepted: 9 Jul 2021 First online: 13 Jul 2021

Academic Editor: Luca Quartuccio Copy Editor: Xi-Jun Chen Production Editor: Xi-Jun Chen

Abstract
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of systemic
autoimmune diseases associated with serum ANCA positivity that affect small to medium vessels with
inflammation and endothelial injury. This group includes several diseases: granulomatosis with polyangiitis (GPA),
microscopic polyangiitis, eosinophilic GPA, and drug-induced AAV. A few AAVs are ANCA negative, but this form
has decreased with the increase in detection methods. Different genetic, epigenetic, and environmental risk factors
contribute to the pathogenesis of AAV. ANCA’s role in the origin of vasculitis has led to a better grasp of the
disease. Research has also improved the treatment, more precisely to tune its intensity, translating into better
outcomes. However, there is still a gap to be filled with new potential and testable biomarkers for diagnosis,
disease activity, and prognosis, which we discuss here.

Keywords: Pathogenesis, ANCA, ANCA-associated vasculitis, biomarkers

INTRODUCTION
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of systemic
autoimmune diseases that affect small to medium vessels with inflammatory and endothelial injury. In the
early 1980s, the first case report mentioning ANCA presence in the serum of patients with segmental
necrotizing glomerulonephritis triggered a new chapter in immune-mediated diseases . The discovery of
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