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

Abstract

BACKGROUND:

Eosinophilic 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 METHODS:

We 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.

RESULTS:

Fifty-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.

CONCLUSION:

These 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.

• Authors: Berengere Molina, Roberto Padoan, Maria Letizia Urban, Pavel Novikov, Marco Caminati, Camille Taillé, Antoine Néel, Laurence Bouillet, Paolo Fraticelli, Nicolas Schleinitz, Christine Christides, Laura Moi, Bertrand Godeau, Ann Knight, Jan Walter Schroeder, Sylvain Marchand-Adam, Helder Gil, Vincent Cottin, Cécile-Audrey Durel, Elena Gelain, Boris Lerais, Marc Ruivard, Matthieu Groh, Maxime Samson, Luca Moroni, Jens Thiel, Anna Kernder, Jan Willem Cohen Tervaert, Giulia Costanzo, Marco Folci, Sonia Rizzello, Pascal Cohen, Giacomo Emmi, Benjamin Terrier
• Journal: Annals of the rheumatic diseases (Ann Rheum Dis) Vol. 82 Issue 12 Pg. 1587-1593 (12 2023) ISSN: 1468-2060 [Electronic] England
• PMID: 37734881 (Publication Type: Multicenter Study, Observational Study, Journal Article)
• Copyright: © Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.

Chemical References

• dupilumab
• Prednisone

Topics

• Humans
• Granulomatosis with Polyangiitis (complications, drug therapy, diagnosis)
• Churg-Strauss Syndrome (complications, drug therapy)
• Retrospective Studies
• Prednisone (therapeutic use)
• Treatment Outcome
• Asthma (drug therapy, complications)
• Eosinophilia (drug therapy, complications)
• Recurrence