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Quartuccio. Vessel Plus 2022;6:23 Vessel Plus
DOI: 10.20517/2574-1209.2021.127

Editorial Open Access

ANCA-associated vasculitis: a new therapeutic area

for precision medicine

Luca Quartuccio
Department of Medicine, Rheumatology, University of Udine, Udine 33100, Italy.

Correspondence to: Prof. Luca Quartuccio, Department of Medicine, University of Udine, Via Colugna 50, Udine 33100, Italy.
E-mail: luca.quartuccio@asufc.sanita.fvg.it

How to cite this article: Quartuccio L. ANCA-associated vasculitis: a new therapeutic area for precision medicine. Vessel Plus
2022;6:23. https://dx.doi.org/10.20517/2574-1209.2021.127

Received: 13 Oct 2021 Accepted: 19 Oct 2021 Published: 8 Apr 2022

Academic Editor: Alexander D. Verin Copy Editor: Yue-Yue Zhang Production Editor: Yue-Yue Zhang

The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are categorized into three
distinct and heterogeneous clinical conditions: granulomatosis with polyangiitis (GPA), microscopic
[1]
polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) .
They are rare autoimmune diseases of unknown aetiology, characterised by inflammation that can cause
necrosis of blood vessels. AAVs are considered small vessel vasculitides but vessels of larger size may be
involved. The discovery of perinuclear and cytoplasmic patterns on indirect immunofluorescence (P-ANCA
and C-ANCA) and the main specificities myeloperoxidase and proteinase 3 (PR3) were recognised in the
1980s . The Chapel Hill Consensus Conference described AAVs in 1994 and the classification was revised
[2]
[1]
in 2012 . This classification, based on clinical phenotype, has been challenged as ethnic studies and
genome-wide association studies clearly support the view of a genetic role in the aetiology of AAV and the
associations with HLA (DQ in MPA), SERPINA1 (in GPA), and PRTN3 (in GPA) were primarily aligned
[3]
with ANCA specificity rather than with clinical definition of GPA or MPA .

Although AAV is a rare disease, recent studies focusing on the healthcare burden of AAV reveal a high level
[4]
of source consumption .

© The Author(s) 2022. Open Access This article is licensed under a Creative Commons Attribution 4.0
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