Dupilumab for relapsing or refractory sinonasal and/or asthma manifestations in eosinophilic granulomatosis with polyangiitis: a European retrospective study

BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is often associated with glucocorticoid-dependent asthma and/or ear, nose and throat (ENT) manifestations. When immunosuppressants and/or mepolizumab are ineffective, dupilumab could be an option. We describe the safety and efficacy of o...

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Published inAnnals of the rheumatic diseases Vol. 82; no. 12; pp. 1587 - 1593
Main Authors Molina, Berengere, Padoan, Roberto, Urban, Maria Letizia, Novikov, Pavel, Caminati, Marco, Taillé, Camille, Néel, Antoine, Bouillet, Laurence, Fraticelli, Paolo, Schleinitz, Nicolas, Christides, Christine, Moi, Laura, Godeau, Bertrand, Knight, Ann, Schroeder, Jan Walter, Marchand-Adam, Sylvain, Gil, Helder, Cottin, Vincent, Durel, Cécile-Audrey, Gelain, Elena, Lerais, Boris, Ruivard, Marc, Groh, Matthieu, Samson, Maxime, Moroni, Luca, Thiel, Jens, Kernder, Anna, Cohen Tervaert, Jan Willem, Costanzo, Giulia, Folci, Marco, Rizzello, Sonia, Cohen, Pascal, Emmi, Giacomo, Terrier, Benjamin
Format Journal Article
LanguageEnglish
Published London BMJ Publishing Group Ltd and European League Against Rheumatism 01.12.2023
BMJ Publishing Group LTD
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Summary:BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is often associated with glucocorticoid-dependent asthma and/or ear, nose and throat (ENT) manifestations. When immunosuppressants and/or mepolizumab are ineffective, dupilumab could be an option. We describe the safety and efficacy of off-label use of dupilumab in relapsing and/or refractory EGPA.Patients and methodsWe conducted an observational multicentre study of EGPA patients treated with dupilumab. Complete response was defined by Birmingham Vasculitis Activity Score (BVAS)=0 and prednisone dose ≤4 mg/day, and partial response by BVAS=0 and prednisone dose >4 mg/day. Eosinophilia was defined as an eosinophil count >500/mm3.ResultsFifty-one patients were included. The primary indication for dupilumab was disabling ENT symptoms in 92%. After a median follow-up of 13.1 months, 18 patients (35%) reported adverse events (AEs), including two serious AEs. Eosinophilia was reported in 34 patients (67%), with a peak of 2195/mm3 (IQR 1268–4501) occurring at 13 weeks (IQR 4–36) and was associated with relapse in 41%. Twenty-one patients (41%) achieved a complete response and 12 (24%) a partial response. Sixteen (31%) patients experienced an EGPA relapse while on dupilumab, which was associated with blood eosinophilia in 14/16 (88%) patients. The median eosinophil count at the start of dupilumab was significantly lower in relapsers than in non-relapsers, as was the median time between stopping anti-IL-5/IL-5R and switching to dupilumab.ConclusionThese results suggest that dupilumab may be effective in treating patients with EGPA-related ENT manifestations. However, EGPA flares occurred in one-third of patients and were preceded by eosinophilia in 88%, suggesting that caution is required.
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ISSN:0003-4967
1468-2060
1468-2060
DOI:10.1136/ard-2023-224756