%0 Multicenter Study
%T Dupilumab for relapsing or refractory sinonasal and/or asthma manifestations in eosinophilic granulomatosis with polyangiitis: a European retrospective study.
%A Molina B
%A Padoan R
%A Urban ML
%A Novikov P
%A Caminati M
%A Taillé C
%A Néel A
%A Bouillet L
%A Fraticelli P
%A Schleinitz N
%A Christides C
%A Moi L
%A Godeau B
%A Knight A
%A Schroeder JW
%A Marchand-Adam S
%A Gil H
%A Cottin V
%A Durel CA
%A Gelain E
%A Lerais B
%A Ruivard M
%A Groh M
%A Samson M
%A Moroni L
%A Thiel J
%A Kernder A
%A Cohen Tervaert JW
%A Costanzo G
%A Folci M
%A Rizzello S
%A Cohen P
%A Emmi G
%A Terrier B
%J Ann Rheum Dis
%V 82
%N 12
%D 2023 12 21
%M 37734881
%F 27.973
%R 10.1136/ard-2023-224756
%X 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.<BR>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.<BR>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.<BR>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.