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

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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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Acknowledgements

The authors would like to thank Professor Clifford E. Roberson for his valuable aid in revising and editing the manuscript.

Funding

MA Alba was supported by the Biomedicine International Training Research program for Excellent Clinician-Scientists (BITRECS), IDIBAPS.

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  1. Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga No. 15, Col. Sección XVI, Tlalpan, 14080, Mexico City, Mexico

    Maricruz Domínguez-Quintana & Andrea Hinojosa-Azaola

  2. Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clínic, IRB-CELLEX, Institut D’Investigacions Biomèdiques August Pi Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain

    Marco A. Alba

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MQD, MAA, and AHA contributed to the conception and design of the work; MQD, MAA, and AHA participated in the acquisition, analysis, and interpretation of the information; MQD, MAA, and AHA drafted the work and revised it critically for important intellectual content; MQD, MAA, and AHA gave final approval of the version to be published and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

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Domínguez-Quintana, M., Alba, M.A. & Hinojosa-Azaola, A. Classification of ANCA-associated vasculitis: differences based on ANCA specificity and clinicopathologic phenotype. Rheumatol Int 41, 1717–1728 (2021). https://doi.org/10.1007/s00296-021-04966-5

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