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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MDPI AG
2023-03-01
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| Series: | Biomedicines |
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| Online Access: | https://www.mdpi.com/2227-9059/11/3/776 |
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| author | Andrea Matucci Emanuele Vivarelli Margherita Perlato Valentina Mecheri Matteo Accinno Lorenzo Cosmi Paola Parronchi Oliviero Rossi Alessandra Vultaggio |
| author_facet | Andrea Matucci Emanuele Vivarelli Margherita Perlato Valentina Mecheri Matteo Accinno Lorenzo Cosmi Paola Parronchi Oliviero Rossi Alessandra Vultaggio |
| author_sort | Andrea Matucci |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-11T06:53:55Z |
| format | Article |
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| institution | Directory Open Access Journal |
| issn | 2227-9059 |
| language | English |
| last_indexed | 2024-03-11T06:53:55Z |
| publishDate | 2023-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomedicines |
| spelling | doaj.art-cba68cd28bb0436794f2472d1f5014eb2023-11-17T09:45:27ZengMDPI AGBiomedicines2227-90592023-03-0111377610.3390/biomedicines11030776EGPA Phenotyping: Not Only ANCA, but Also EosinophilsAndrea Matucci0Emanuele Vivarelli1Margherita Perlato2Valentina Mecheri3Matteo Accinno4Lorenzo Cosmi5Paola Parronchi6Oliviero Rossi7Alessandra Vultaggio8Immunoallergology Unit, Careggi University Hospital, Largo Brambilla 3, 50134 Florence, ItalyImmunology and Cellular Therapy Unit, Department of Experimental and Clinical Medicine, Careggi University Hospital, 50134 Florence, ItalyImmunology and Cellular Therapy Unit, Department of Experimental and Clinical Medicine, Careggi University Hospital, 50134 Florence, ItalyImmunology and Cellular Therapy Unit, Department of Experimental and Clinical Medicine, Careggi University Hospital, 50134 Florence, ItalyImmunoallergology Unit, Careggi University Hospital, Largo Brambilla 3, 50134 Florence, ItalyImmunoallergology Unit, Department of Experimental and Clinical Medicine, Careggi University Hospital, 50134 Florence, ItalyImmunology and Cellular Therapy Unit, Department of Experimental and Clinical Medicine, Careggi University Hospital, 50134 Florence, ItalyImmunoallergology Unit, Careggi University Hospital, Largo Brambilla 3, 50134 Florence, ItalyImmunoallergology Unit, Department of Experimental and Clinical Medicine, Careggi University Hospital, 50134 Florence, ItalyBackground: 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.https://www.mdpi.com/2227-9059/11/3/776EGPAeosinophilsvasculitisphenotypeANCA |
| spellingShingle | Andrea Matucci Emanuele Vivarelli Margherita Perlato Valentina Mecheri Matteo Accinno Lorenzo Cosmi Paola Parronchi Oliviero Rossi Alessandra Vultaggio EGPA Phenotyping: Not Only ANCA, but Also Eosinophils Biomedicines EGPA eosinophils vasculitis phenotype ANCA |
| 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 | EGPA eosinophils vasculitis phenotype ANCA |
| url | https://www.mdpi.com/2227-9059/11/3/776 |
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