Session Type: ACR Abstract Session
Session Time: 2:30PM-4:00PM
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg–Strauss syndrome), is characterized by small-vessel necrotizing vasculitis, and blood and tissue eosinophilia in asthmatic individuals. Glucocorticoids (GCs) usually control EGPA, but vasculitis relapses and GC-dependent asthma are frequent, as are long-term adverse events, leading to potential biotherapy use. We examined off-label biological therapy use for relapsing/refractory EGPA.
This retrospective European collaborative study included patients with EGPA, meeting the ACR criteria and/or Chapel Hill Consensus Conference definitions. Treatment efficacy and safety were recorded. Remission was defined as the absence of asthma, sinonasal and vasculitis manifestations with ≤5 mg/day of prednisone, and partial response as the absence of manifestations but requiring 6–10 mg/day of prednisone.
Among the147 patients (74 men, 73 women; median age 52 years) included, 63 (43%) received rituximab (RTX), 51 (35%) mepolizumab (MEPO), at monthly respective doses of 100 mg or 300 mg for 29 (57%) and 22 (43%), and 33 (22%) omalizumab (OMA). Previous treatments were: GCs for all, azathioprine (68%), cyclophosphamide (40%), methotrexate (29%) or mycophenolate mofetil (15%).
At inclusion, median (interquartile range) BVAS in the RTX, OMA and MEPO groups, respectively, were 8.5 (5-13), 2 (2-5) and 2 (2-6). In the RTX-treated patients, median BVAS fell to 1 (0-4.5) at 6 and 0 (0-2) ay 12 months.A Median GCs dose decreased to 7.5 (5-10) at 6 months and 12 months. Overall, remissions, partial responses, therapeutic failure and stop for adverse event, respectively, were noted in 49%, 24%, 24% and 3% for RTX recipients. Remission was observed in 57% of ANCA-positive patients compared to 42% in ANCA-negative patients.
To treat GC-dependent asthma, MEPO had a much better GCs-sparing effect than OMA, and a better overall response. Remissions, partial responses, therapeutic failure and stop for adverse event, respectively, were noted in 15%, 33%, 48% and 4% for OMA recipients and 78%, 10%, 8% and 4% for MEPO recipients.Finally, no obvious difference was noted between patients receiving MEPO 100 mg and those 300 mg monthly, in terms of GC-sparing effect and overall response.
Sixteen (25%) patients stopped RTX: 2 for adverse events, and 14 for refractory disease. Also, 17 (27%) experienced adverse events, mainly severe infections. Seventeen (52%) stopped OMA: 1 for severe infusion reaction, and 16 for refractory disease. Four (12%) patients receiving OMA experienced mild to moderate adverse events. Three (6%) patients stopped MEPO: 2 for adverse events (one severe infusion reaction and one because of paraesthesia), and 1 for pregnancy. Eleven (22%) patients receiving MEPO experienced mild to moderate adverse events, mainly asthenia.
Conclusion: These results suggest that RTX could be effective for 50% of patients with EGPA vasculitis relapses, with an acceptable safety profile. MEPO is highly effective with a good GCs-sparing effect and safety profile in patients with GCs-dependant asthma, and 100 mg monthly seems to be an acceptable dose at first-line.
To cite this abstract in AMA style:Canzian A, Venhoff N, Sartorelli S, Ruppert A, Groh M, Taille C, Rieu V, Smets P, Maurier F, Girszyn N, Samson M, de Moreuil C, Pugnet G, Delbrel X, Kahn J, Puéchal for the French Vasculitis Study Group X, Emmi G, Guillevin L, Dagna L, Thiel J, Vaglio A, Terrier B. Off-Label Use of Biotherapies to Treat Relapsing And/or Refractory Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss) [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/off-label-use-of-biotherapies-to-treat-relapsing-and-or-refractory-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss/. Accessed April 13, 2021.
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