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EGPA Phenotyping: Not Only ANCA, but Also Eosinophils

Background: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a small-vessel necrotizing vasculitis. The anti-neutrophil cytoplasmic antibodies’ (ANCA) role in defining clinical EGPA phenotypes is well established. Although the role of eosinophils in disease pathogenesis has been clearly demon...

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Autores principales: Matucci, Andrea, Vivarelli, Emanuele, Perlato, Margherita, Mecheri, Valentina, Accinno, Matteo, Cosmi, Lorenzo, Parronchi, Paola, Rossi, Oliviero, Vultaggio, Alessandra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10045549/
https://www.ncbi.nlm.nih.gov/pubmed/36979755
http://dx.doi.org/10.3390/biomedicines11030776
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author Matucci, Andrea
Vivarelli, Emanuele
Perlato, Margherita
Mecheri, Valentina
Accinno, Matteo
Cosmi, Lorenzo
Parronchi, Paola
Rossi, Oliviero
Vultaggio, Alessandra
author_facet Matucci, Andrea
Vivarelli, Emanuele
Perlato, Margherita
Mecheri, Valentina
Accinno, Matteo
Cosmi, Lorenzo
Parronchi, Paola
Rossi, Oliviero
Vultaggio, Alessandra
author_sort Matucci, Andrea
collection PubMed
description Background: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a small-vessel necrotizing vasculitis. The anti-neutrophil cytoplasmic antibodies’ (ANCA) role in defining clinical EGPA phenotypes is well established. Although the role of eosinophils in disease pathogenesis has been clearly demonstrated, the value of blood eosinophil count (BEC) as a biomarker of disease phenotypes is currently uncertain. Methods: We retrospectively analyzed EGPA patients referred to our Immunology Clinic. Demographic, laboratory and clinical features were retrieved from clinical records, and a Logistic Regression was fitted to evaluate the predictive power of all baseline clinical and laboratory features to define EGPA phenotypes. Results: 168 patients were recruited. BEC ≤ 1500 cells/mL was predictive of a clinical involvement characterized by asthma, chronic rhinosinusitis with nasal polyps (CRSwNP) and lung opacities (OR 0.18, 95% CI 0.07–0.43; respiratory-limited phenotype); BEC > 3500/mL was predictive of extrapulmonary organ involvement (OR 3.5, 95% CI 1.7–7.1; systemic phenotype). BEC was also predictive of peripheral nervous system (PNS) involvement, with a positive trend with increasing BEC (<1500/mL: OR 0.17, 95%CI, 0.06–0.47; >3500/mL: OR 2.8, 95% CI, 1.5–5.28). ANCA positivity was also predictive of extrapulmonary involvement (OR 4.7, 95% CI 1.9–11.99). Conclusions: according to BEC and irrespective of the ANCA status, two EGPA phenotypes could be identified, named systemic and respiratory-limited phenotypes, with different organ involvement and possibly different prognoses.
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spelling pubmed-100455492023-03-29 EGPA Phenotyping: Not Only ANCA, but Also Eosinophils Matucci, Andrea Vivarelli, Emanuele Perlato, Margherita Mecheri, Valentina Accinno, Matteo Cosmi, Lorenzo Parronchi, Paola Rossi, Oliviero Vultaggio, Alessandra Biomedicines Article Background: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a small-vessel necrotizing vasculitis. The anti-neutrophil cytoplasmic antibodies’ (ANCA) role in defining clinical EGPA phenotypes is well established. Although the role of eosinophils in disease pathogenesis has been clearly demonstrated, the value of blood eosinophil count (BEC) as a biomarker of disease phenotypes is currently uncertain. Methods: We retrospectively analyzed EGPA patients referred to our Immunology Clinic. Demographic, laboratory and clinical features were retrieved from clinical records, and a Logistic Regression was fitted to evaluate the predictive power of all baseline clinical and laboratory features to define EGPA phenotypes. Results: 168 patients were recruited. BEC ≤ 1500 cells/mL was predictive of a clinical involvement characterized by asthma, chronic rhinosinusitis with nasal polyps (CRSwNP) and lung opacities (OR 0.18, 95% CI 0.07–0.43; respiratory-limited phenotype); BEC > 3500/mL was predictive of extrapulmonary organ involvement (OR 3.5, 95% CI 1.7–7.1; systemic phenotype). BEC was also predictive of peripheral nervous system (PNS) involvement, with a positive trend with increasing BEC (<1500/mL: OR 0.17, 95%CI, 0.06–0.47; >3500/mL: OR 2.8, 95% CI, 1.5–5.28). ANCA positivity was also predictive of extrapulmonary involvement (OR 4.7, 95% CI 1.9–11.99). Conclusions: according to BEC and irrespective of the ANCA status, two EGPA phenotypes could be identified, named systemic and respiratory-limited phenotypes, with different organ involvement and possibly different prognoses. MDPI 2023-03-03 /pmc/articles/PMC10045549/ /pubmed/36979755 http://dx.doi.org/10.3390/biomedicines11030776 Text en © 2023 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 Article
Matucci, Andrea
Vivarelli, Emanuele
Perlato, Margherita
Mecheri, Valentina
Accinno, Matteo
Cosmi, Lorenzo
Parronchi, Paola
Rossi, Oliviero
Vultaggio, Alessandra
EGPA Phenotyping: Not Only ANCA, but Also Eosinophils
title EGPA Phenotyping: Not Only ANCA, but Also Eosinophils
title_full EGPA Phenotyping: Not Only ANCA, but Also Eosinophils
title_fullStr EGPA Phenotyping: Not Only ANCA, but Also Eosinophils
title_full_unstemmed EGPA Phenotyping: Not Only ANCA, but Also Eosinophils
title_short EGPA Phenotyping: Not Only ANCA, but Also Eosinophils
title_sort egpa phenotyping: not only anca, but also eosinophils
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10045549/
https://www.ncbi.nlm.nih.gov/pubmed/36979755
http://dx.doi.org/10.3390/biomedicines11030776
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