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Rheumatology International
Published in: Rheumatology International 10/2021

01-10-2021 | ANCA-Associated Vasculitis | Review

Classification of ANCA-associated vasculitis: differences based on ANCA specificity and clinicopathologic phenotype

Authors: Maricruz Domínguez-Quintana, Marco A. Alba, Andrea Hinojosa-Azaola

Published in: Rheumatology International | Issue 10/2021

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Abstract

The classification of vasculitis according to a schema with universal acceptance is challenging, given the heterogeneous and protean nature of these diseases. Formal nomenclature and classification criteria for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have suffered several changes since their first description; none provides comprehensive diagnostic and classification criteria. Different factors account for the difficulties in the classification of vasculitis, including the incomplete understanding of the pathogenesis, the multisystemic nature of the disease, the non-specific patterns of vascular involvement, the overlap between entities, and the presence of various classification systems. The present article reviews the classification of AAV considering different points of view, including clinical, serologic, pathogenetic, organ predilection, therapeutic, and prognostic factors, and provides perspectives on future challenges in the understanding of AAV. There is an unmet need for a unifying view of the disease spectrum that considers the constantly evolving paradigms.
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Metadata
Title
Classification of ANCA-associated vasculitis: differences based on ANCA specificity and clinicopathologic phenotype
Authors
Maricruz Domínguez-Quintana
Marco A. Alba
Andrea Hinojosa-Azaola
Publication date
01-10-2021
Publisher
Springer Berlin Heidelberg
Published in
Rheumatology International / Issue 10/2021
Print ISSN: 0172-8172
Electronic ISSN: 1437-160X
DOI
https://doi.org/10.1007/s00296-021-04966-5

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