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.

Dupilumab for relapsing or refractory sinonasal and/or asthma manifestations in eosinophilic granulomatosis with polyangiitis: a European retrospective study / 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. - In: ANNALS OF THE RHEUMATIC DISEASES. - ISSN 0003-4967. - ELETTRONICO. - (2023), pp. ard-2023-224756-ard-2023-224756. [10.1136/ard-2023-224756]

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

Urban, Maria Letizia;Gelain, Elena;Emmi, Giacomo;
2023

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.
2023
ard-2023-224756
ard-2023-224756
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
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in FLORE sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1333256
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 10
  • ???jsp.display-item.citation.isi??? 8
social impact