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Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterised by eosinophilic (eg, respiratory involvement, cardiomyopathy, gastroenteritis) and vasculitic manifestations (eg, neuropathy, glomerulonephritis).1
Rituximab is an established treatment in granulomatosis with polyangiitis and microscopic polyangiitis, and growing evidence indicates that it seems effective also in EGPA, mainly to induce and maintain remission of vasculitic involvement.2 3 However, its efficacy on respiratory manifestations seems limited. Conversely, the anti-IL5 mepolizumab, recently licensed for relapsing-refractory EGPA, is effective on respiratory manifestations, although it may also partially control systemic ones.3–5
Based on the idea that combining treatments with complementary mechanisms of action might induce and maintain remission of both disease components,6 7 we investigated the efficacy and safety of a regimen based on sequential rituximab and mepolizumab for the control of EGPA.
This multicentre, European, retrospective study included patients meeting the American College of Rheumatology classification criteria for EGPA or the eligibility criteria proposed in the MIRRA trial.1 Only patients who received therapy with rituximab (any dosage), and subsequent treatment with mepolizumab (100–300 mg/4 weeks) within 12 months from last rituximab administration, without other induction/maintenance therapies in the meanwhile, were included.
Treatment efficacy was assessed considering disease activity (by the Birmingham Vasculitis Activity Score, BVAS), eosinophil count and glucocorticoid dose.1 Asthma attacks and adverse events (AEs) were also assessed.
The study received ethical approval (University of Florence IRB; ref.16821_OSS); as this …
DP, AV and GE are joint senior authors.
Handling editor Josef S Smolen
AB and MLU contributed equally.
Collaborators The manuscript is submitted on behalf on the European EGPA Study Group. Collaborators (in alphabetical order): Paolo Cameli (University of Siena, Siena, Italy), Fabrizio Conti (Sapienza University, Rome, Italy), Filippo Fagni (University of Florence, Florence, Italy and University Hospital Erlangen, Erlangen, Germany), Laura Losappio (Niguarda Hospital, Milan, Italy), Danilo Malandrino (University of Florence, Florence, Italy), Matteo Mazzetti (Careggi University Hospital, Florence, Italy), Ruggero Mazzotta (University of Florence, Florence, Italy), Luca Moroni (IRCCS Ospedale San Raffaele, Milan, and Vita-Salute San Raffaele University – Milan, Italy), Adalgisa Palermo (University of Florence, Florence, Italy), Camillo Ribi (University of Lausanne, Lausanne, Switzerland), Franco Schiavon (University of Padua, Padua, Italy), Elena Silvestri (University of Florence, Florence, Italy), Benjamin Terrier (Hôpital Cochin and Université de Paris, Paris, France), Paola Toniati (ASST Spedali Civili, Brescia, Italy)
Contributors All people who contributed to this work are listed as coauthors or collaborators.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests GE received consultation honoraria from GSK outside the current work. LD received consultation honoraria from GSK outside the current work. DJ’s disclosures of commercial conflicts are as follows: AstraZeneca, Aurinia, BMS, Boehringer-Ingelheim, Chemocentryx, Chugai, CSL, GSK, Infla-RX, Janssen, Novartis, Roche/Genentech, Takeda and Vifor. LM received consultation honoraria from GSK outside the current work. PP received consultation honoraria from GSK, and Novartisand LEOPharma. Professor Camillo Ribi received consultation honoraria from GSK outside the current work. JS received Advisory Board fees from AstraZeneca and GSK. AV received consultation honoraria from GSK outside the current work. All other authors and collaborators declare no conflicts of interest.
Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.
Provenance and peer review Not commissioned; externally peer reviewed.