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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.
Dupilumab for relapsing or refractory sinonasal and/or asthma manifestations in eosinophilic granulomatosis with polyangiitis: a European retrospective study.
Ann Rheum Dis. 2023; 82(12):1587-1593 Doi: 10.1136/ard-2023-224756
Web of Science PubMed FullText FullText_MUG

Co-authors Med Uni Graz: Thiel Jens
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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.
Find related publications in this database (using NLM MeSH Indexing): Humans - administration & dosage; Granulomatosis with Polyangiitis - complications, drug therapy, diagnosis; Churg-Strauss Syndrome - complications, drug therapy; Retrospective Studies - administration & dosage; Prednisone - therapeutic use; Treatment Outcome - administration & dosage; Asthma - drug therapy, complications; Eosinophilia - drug therapy, complications; Recurrence - administration & dosage
Find related publications in this database (Keywords): Systemic vasculitis; Immune System Diseases; Biological Therapy; Autoimmune Diseases; Therapeutics