Cargando…

Type of ANCA May Be Indispensable in Distinguishing Subphenotypes of Different Clinical Entities in ANCA-Associated Vasculitis

SIMPLE SUMMARY: ANCA specificity seems to correlate more accurately with the symptomatology and disease outcome in ANCA-associated vasculitis in comparison to the classification based on clinical syndromes, as described in the Chapel Hill Consensus. This review analyzes the main differences in epide...

Descripción completa

Detalles Bibliográficos
Autores principales: Konstantouli, Afroditi Maria, Lioulios, Georgios, Stai, Stamatia, Moysidou, Eleni, Fylaktou, Asimina, Papagianni, Aikaterini, Stangou, Maria
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9604609/
https://www.ncbi.nlm.nih.gov/pubmed/36294902
http://dx.doi.org/10.3390/life12101467
_version_ 1784817857021870080
author Konstantouli, Afroditi Maria
Lioulios, Georgios
Stai, Stamatia
Moysidou, Eleni
Fylaktou, Asimina
Papagianni, Aikaterini
Stangou, Maria
author_facet Konstantouli, Afroditi Maria
Lioulios, Georgios
Stai, Stamatia
Moysidou, Eleni
Fylaktou, Asimina
Papagianni, Aikaterini
Stangou, Maria
author_sort Konstantouli, Afroditi Maria
collection PubMed
description SIMPLE SUMMARY: ANCA specificity seems to correlate more accurately with the symptomatology and disease outcome in ANCA-associated vasculitis in comparison to the classification based on clinical syndromes, as described in the Chapel Hill Consensus. This review analyzes the main differences in epidemiology, pathogenesis, histological and clinical manifestations and treatment response according to ANCA type. ABSTRACT: The traditional nomenclature system for classifying antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) based on clinical phenotype describes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA) as distinct clinical entities. This classification has proved its expedience in clinical trials and everyday clinical practice; yet, a substantial overlap in clinical presentation still exists and often causes difficulties in prompt definition and clinical distinction. Additionally, new insights into the AAV pathogenesis point out that PR3 and MPO-AAV may not represent expressions of the same disease spectrum but rather two distinct disorders, as they display significant differences. Thus, it is supported that a classification based on ANCA serotype (PR3-ANCA, MPO-ANCA or ANCA-negative) could be more accurate and also closer to the nature of the disease compared to the phenotype-based one. This review aims to elucidate the major differences between PR3 and MPO-AAV in terms of epidemiology, pathogenesis, histological and clinical manifestations and response to therapeutic approaches.
format Online
Article
Text
id pubmed-9604609
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-96046092022-10-27 Type of ANCA May Be Indispensable in Distinguishing Subphenotypes of Different Clinical Entities in ANCA-Associated Vasculitis Konstantouli, Afroditi Maria Lioulios, Georgios Stai, Stamatia Moysidou, Eleni Fylaktou, Asimina Papagianni, Aikaterini Stangou, Maria Life (Basel) Review SIMPLE SUMMARY: ANCA specificity seems to correlate more accurately with the symptomatology and disease outcome in ANCA-associated vasculitis in comparison to the classification based on clinical syndromes, as described in the Chapel Hill Consensus. This review analyzes the main differences in epidemiology, pathogenesis, histological and clinical manifestations and treatment response according to ANCA type. ABSTRACT: The traditional nomenclature system for classifying antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) based on clinical phenotype describes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA) as distinct clinical entities. This classification has proved its expedience in clinical trials and everyday clinical practice; yet, a substantial overlap in clinical presentation still exists and often causes difficulties in prompt definition and clinical distinction. Additionally, new insights into the AAV pathogenesis point out that PR3 and MPO-AAV may not represent expressions of the same disease spectrum but rather two distinct disorders, as they display significant differences. Thus, it is supported that a classification based on ANCA serotype (PR3-ANCA, MPO-ANCA or ANCA-negative) could be more accurate and also closer to the nature of the disease compared to the phenotype-based one. This review aims to elucidate the major differences between PR3 and MPO-AAV in terms of epidemiology, pathogenesis, histological and clinical manifestations and response to therapeutic approaches. MDPI 2022-09-21 /pmc/articles/PMC9604609/ /pubmed/36294902 http://dx.doi.org/10.3390/life12101467 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Konstantouli, Afroditi Maria
Lioulios, Georgios
Stai, Stamatia
Moysidou, Eleni
Fylaktou, Asimina
Papagianni, Aikaterini
Stangou, Maria
Type of ANCA May Be Indispensable in Distinguishing Subphenotypes of Different Clinical Entities in ANCA-Associated Vasculitis
title Type of ANCA May Be Indispensable in Distinguishing Subphenotypes of Different Clinical Entities in ANCA-Associated Vasculitis
title_full Type of ANCA May Be Indispensable in Distinguishing Subphenotypes of Different Clinical Entities in ANCA-Associated Vasculitis
title_fullStr Type of ANCA May Be Indispensable in Distinguishing Subphenotypes of Different Clinical Entities in ANCA-Associated Vasculitis
title_full_unstemmed Type of ANCA May Be Indispensable in Distinguishing Subphenotypes of Different Clinical Entities in ANCA-Associated Vasculitis
title_short Type of ANCA May Be Indispensable in Distinguishing Subphenotypes of Different Clinical Entities in ANCA-Associated Vasculitis
title_sort type of anca may be indispensable in distinguishing subphenotypes of different clinical entities in anca-associated vasculitis
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9604609/
https://www.ncbi.nlm.nih.gov/pubmed/36294902
http://dx.doi.org/10.3390/life12101467
work_keys_str_mv AT konstantouliafroditimaria typeofancamaybeindispensableindistinguishingsubphenotypesofdifferentclinicalentitiesinancaassociatedvasculitis
AT liouliosgeorgios typeofancamaybeindispensableindistinguishingsubphenotypesofdifferentclinicalentitiesinancaassociatedvasculitis
AT staistamatia typeofancamaybeindispensableindistinguishingsubphenotypesofdifferentclinicalentitiesinancaassociatedvasculitis
AT moysidoueleni typeofancamaybeindispensableindistinguishingsubphenotypesofdifferentclinicalentitiesinancaassociatedvasculitis
AT fylaktouasimina typeofancamaybeindispensableindistinguishingsubphenotypesofdifferentclinicalentitiesinancaassociatedvasculitis
AT papagianniaikaterini typeofancamaybeindispensableindistinguishingsubphenotypesofdifferentclinicalentitiesinancaassociatedvasculitis
AT stangoumaria typeofancamaybeindispensableindistinguishingsubphenotypesofdifferentclinicalentitiesinancaassociatedvasculitis