Introduction: Data showing effectiveness of mepolizumab in patients with eosinophilic granulomatosis with polyangiitis (EGPA) are limited. Methods: This is a single-center retrospective chart review of patients with EGPA treated with mepolizumab. Clinical, laboratory, functional parameters and asthma, rhinitis control, and quality of life scores (Asthma Control Test [ACT], Asthma Quality of Life Questionnaire [AQLQ], Rhinoconjunctivitis Quality of Life Questionnaire [RQLQ], and SinoNasal Outcome Test [SNOT]-22) were evaluated at the baseline, 6th month, and 12th month. Complete response was defined as the absence of asthma and/or ear, nasal symptoms and exacerbations with a prednisone of ≤7.5 mg/day, partial response if it was achieved with a prednisone of >7.5 mg/day. Results: Overall, 25 patients (18 F/7 M) with a median age of 47 years (23–76) were enrolled. Mepolizumab 100 mg/month was administered (dose increased to 300 mg/month in 3 patients). Mepolizumab significantly decreased daily dose of oral corticosteroid (OCS) from 11.04 mg to 3.65 mg together with a significant improvement in ACT, AQLQ, RQLQ, and SNOT-22 scores and a significant reduction in asthma exacerbations and blood eosinophil count at the 6th and 12th month (all p values <0.05). The mean forced expiratory volume in 1 s increased (at baseline: 1.88 L to 2.46 L at the 12th month [p = 0.037]). Seventy-six percent of patients responded completely at the 6th month and 81.25% at the 12th month. The complete responders at the 6th and 12th month were older than partial responders and nonresponders (p = 0.030 and p = 0.057, respectively). Patients with complete response at the 6th month were on lower doses of OCS than partial responders and nonresponders (p = 0.029). Conclusions: Low-dose mepolizumab was effective in EGPA patients by improving sinonasal and asthma outcomes, while reducing the need for OCS.

Keywords:
Eosinophilic granulomatosis with polyangiitis, Mepolizumab, Asthma, Vasculitis, Treatment response
1.
Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, et al. Churg-Strauss syndrome.
Autoimmun Rev
. 2015;14(4):341–8.
2.
Raffray L, Guillevin L. Updates for the treatment of EGPA.
Presse Med
. 2020;49(3):104036.
3.
Pavord ID, Bel EH, Bourdin A, Chan R, Han JK, Keene ON, et al. From DREAM to REALITI-A and beyond: mepolizumab for the treatment of eosinophil-driven diseases.
Allergy
. 2022;77(3):778–97.
4.
Isozaki T, Homma T, Sagara H, Kasama T. Role of cytokines in EGPA and the possibility of treatment with an anti-IL-5 antibody.
J Clin Med
. 2020;9(12):3890.
5.
Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, et al. Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis.
N Engl J Med
. 2017;376(20):1921–32.
6.
Vergles M, Matković Z, Lalić K, Trkanjec JT, Tudorić N. Mepolizumab as a glucocorticoid-sparing agent in eosinophilic granulomatosis with polyangiitis (EGPA): is a lower dose sufficient?
J Asthma
. 2021;58(12):1675–9.
7.
Vultaggio A, Nencini F, Bormioli S, Vivarelli E, Dies L, Rossi O, et al. Low-dose mepolizumab effectiveness in patients suffering from eosinophilic granulomatosis with polyangiitis.
Allergy Asthma Immunol Res
. 2020;12(5):885–93.
8.
Detoraki A, Tremante E, Poto R, Morelli E, Quaremba G, Granata F, et al. Real-life evidence of low-dose mepolizumab efficacy in EGPA: a case series.
Respir Res
. 2021;22(1):185.
9.
Celebi Sozener Z, Gorgulu B, Mungan D, Sin BA, Misirligil Z, Aydin O, et al. Omalizumab in the treatment of eosinophilic granulomatosis with polyangiitis (EGPA): single-center experience in 18 cases.
World Allergy Organ J
. 2018;11(1):39.
10.
Özdel Öztürk B, Yavuz Z, Eraslan D, Mungan D, Demirel YS, Aydın Ö, et al. Mepolizumab is an effective option in severe eosinophilic asthma regardless of baseline features: single-center real-life data.
Int Arch Allergy Immunol
. 2022;183(5):526–38.
11.
Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).
Arthritis Rheum
. 1990;33(8):1094–100.
12.
Global Initiative for Asthma.
Global strategy for asthma management and prevention
. Fontana, WI: Global Initiative for Asthma; 2021. Available from: www.ginasthma.org.
13.
Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management.
Eur J Intern Med
. 2015;26(7):545–53.
14.
Jachiet M, Samson M, Cottin V, Kahn JE, Le Guenno G, Bonniaud P, et al. Anti-IgE monoclonal antibody (omalizumab) in refractory and relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss): data on seventeen patients.
Arthritis Rheumatol
. 2016;68(9):2274–82.
15.
Tsurikisawa N, Oshikata C, Watanabe M, Fukuda N, Yamaguchi T, Kiyohara H, et al. Clinical features of patients with active eosinophilic granulomatosis with polyangiitis successfully treated with mepolizumab.
Int Arch Allergy Immunol
. 2021;182(8):744–56.
16.
Ueno M, Miyagawa I, Nakano K, Iwata S, Hanami K, Fukuyo S, et al. Effectiveness and safety of mepolizumab in combination with corticosteroids in patients with eosinophilic granulomatosis with polyangiitis.
Arthritis Res Ther
. 2021;23(1):86.
17.
Canzian A, Venhoff N, Urban ML, Sartorelli S, Ruppert AM, Groh M, et al. Use of biologics to treat relapsing and/or refractory eosinophilic granulomatosis with polyangiitis: data from a European Collaborative Study.
Arthritis Rheumatol
. 2021;73(3):498–503.
18.
Bettiol A, Urban ML, Dagna L, Cottin V, Franceschini F, Del Giacco S, et al. Mepolizumab for eosinophilic granulomatosis with polyangiitis: a European Multicenter Observational study.
Arthritis Rheumatol
. 2022;74(2):295–306.
19.
Steinfeld J, Bradford ES, Brown J, Mallett S, Yancey SW, Akuthota P, et al. Evaluation of clinical benefit from treatment with mepolizumab for patients with eosinophilic granulomatosis with polyangiitis.
J Allergy Clin Immunol
. 2019;143(6):2170–7.
20.
Furuta S, Iwamoto T, Nakajima H. Update on eosinophilic granulomatosis with polyangiitis.
Allergol Int
. 2019;68(4):430–6.
21.
Caminati M, Crisafulli E, Lunardi C, Micheletto C, Festi G, Maule M, et al. Mepolizumab 100 mg in severe asthmatic patients with EGPA in remission phase.
J Allergy Clin Immunol Pract
. 2021;9(3):1386–8.
22.
Ríos-Garcés R, Prieto-González S, Hernández-Rodríguez J, Arismendi E, Alobid I, Penatti AE, et al. Response to mepolizumab according to disease manifestations in patients with eosinophilic granulomatosis with polyangiitis.
Eur J Intern Med
. 2022;95:61–6.
23.
Ueno M, Miyagawa I, Aritomi T, Kimura K, Iwata S, Hanami K, et al. Safety and effectiveness of mepolizumab therapy in remission induction therapy for eosinophilic granulomatosis with polyangiitis: a retrospective study.
Arthritis Res Ther
. 2022;24(1):159.
24.
Watts RA, Lane SE, Scott DG, Koldingsnes W, Nossent H, Gonzalez-Gay MA, et al. Epidemiology of vasculitis in Europe.
Ann Rheum Dis
. 2001;60(12):1156–7.
© 2022 S. Karger AG, Basel
2022
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.