Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis (EGPA): a European multicenter observational study

Publication

Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis (EGPA): a European multicenter observational study

Journal Paper/Review - Aug 4, 2021

European EGPA Study Group, Moroni Luca, Fagni Filippo, Bello Federica, Fiori Davide, Ribi Camillo, Cohen Tervaert Jan Willem, Bargagli Elena, Samson Maxime, Hellmich Bernhard, Mohammad Aladdin J, Vacca Angelo, Roccatello Dario, Ramirez Giuseppe Alvise, Nasser Mouhamad, Emmi Giacomo, Vaglio Augusto, Prisco Domenico, Jayne David, Salvarani Carlo, Iannone Florenzo, Seeliger Benjamin, Egan Allyson, Padoan Roberto, Firinu Davide, Toniati Paola, Marvisi Chiara, Fraticelli Paolo, Cinetto Francesco, Berti Alvise, Lombardi Carlo, Baldini Chiara, Novikov Pavel, Lopalco Giuseppe, Neumann Thomas, Schiavon Franco, Del Giacco Stefano, Franceschini Franco, Cottin Vincent, Dagna Lorenzo, Urban Maria Letizia, Alberici Federico, Folci Marco, Crimi Claudia, Silvagni Ettore, Monti Sara, Kernder Anna Luise, Schroeder Jan, Espígol-Frigolé Georgina, Moosig Frank, Parronchi Paola, Lunardi Claudio, Quartuccio Luca, Sinico Renato Alberto, Negrini Simone, Bettiol Alessandra

Citation

European EGPA Study Group, Moroni L, Fagni F, Bello F, Fiori D, Ribi C, Cohen Tervaert J, Bargagli E, Samson M, Hellmich B, Mohammad A, Vacca A, Roccatello D, Ramirez G, Nasser M, Emmi G, Vaglio A, Prisco D, Jayne D, Salvarani C, Iannone F, Seeliger B, Egan A, Padoan R, Firinu D, Toniati P, Marvisi C, Fraticelli P, Cinetto F, Berti A, Lombardi C, Baldini C, Novikov P, Lopalco G, Neumann T, Schiavon F, Del Giacco S, Franceschini F, Cottin V, Dagna L, Urban M, Alberici F, Folci M, Crimi C, Silvagni E, Monti S, Kernder A, Schroeder J, Espígol-Frigolé G, Moosig F, Parronchi P, Lunardi C, Quartuccio L, Sinico R, Negrini S, Bettiol A. Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis (EGPA): a European multicenter observational study. Arthritis Rheumatol 2021

Type

Journal Paper/Review (English)

Journal

Arthritis Rheumatol 2021

Publication Date

Aug 4, 2021

Issn Electronic

2326-5205

Brief description/objective

OBJECTIVE
Mepolizumab proved efficacious for eosinophilic granulomatosis with polyangiitis (EGPA, former Churg-Strauss) at the dosage of 300mg/4 weeks in the randomized controlled MIRRA trial. Few successful real-life experiences with the dosage approved for severe eosinophilic asthma (100mg/4 weeks) were recently reported. We retrospectively assessed the effectiveness and safety of mepolizumab 100 and 300mg/4 weeks in a large European EGPA cohort.

METHODS
We included all EGPA patients treated with mepolizumab at the recruiting centres in 2015-2020. Treatment response was evaluated from month 3 through 24 (T3-T24) after mepolizumab starting. Complete response (CR) was defined as no disease activity (Birmingham Vasculitis Activity Score, BVAS=0) and a prednisone dose ≤4mg/day. Respiratory outcomes included asthma and ear-nose-throat (ENT) exacerbations.

RESULTS
We included 203 patients, of whom 191 at stable dosage (158 mepolizumab 100mg/4 weeks, 33 300mg/4 weeks). At T3, 25 patients (12.3%) had a CR. CR rates increased to 30.4% and 35.7% at T12 and T24 and were comparable between mepolizumab 100 and 300mg/4 weeks. Mepolizumab led to a significant reduction in BVAS, prednisone dose, eosinophil counts from T3 through T24, with no significant differences between 100 and 300 mg/4weeks. Eighty-two patients (40.4%) experienced asthma exacerbations [57/158 (36%) on 100mg/4 weeks; 17/33 (52%) on 300mg/4 weeks]. Thirty-one (15.3%) experienced ENT exacerbations. Forty-four patients (21.7%) experienced adverse events, most being non-serious (38/44).

CONCLUSION
Mepolizumab both at 100 and 300mg/4 weeks is effective for EGPA. The two dosages should be compared in the setting of a controlled trial.