{Reference Type}: Multicenter Study
{Title}: Dupilumab for relapsing or refractory sinonasal and/or asthma manifestations in eosinophilic granulomatosis with polyangiitis: a European retrospective study.
{Author}: Molina B;Padoan R;Urban ML;Novikov P;Caminati M;Taillé C;Néel A;Bouillet L;Fraticelli P;Schleinitz N;Christides C;Moi L;Godeau B;Knight A;Schroeder JW;Marchand-Adam S;Gil H;Cottin V;Durel CA;Gelain E;Lerais B;Ruivard M;Groh M;Samson M;Moroni L;Thiel J;Kernder A;Cohen Tervaert JW;Costanzo G;Folci M;Rizzello S;Cohen P;Emmi G;Terrier B;
{Journal}: Ann Rheum Dis
{Volume}: 82
{Issue}: 12
{Year}: 2023 12 21
{Factor}: 27.973
{DOI}: 10.1136/ard-2023-224756
{Abstract}: 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.