Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg–Strauss) is characterized by pulmonary and systemic small-vessel necrotizing vasculitis, vascular and/or extravascular granulomas, eosinophilia and tissue infiltration by eosinophils, occurring in individuals with asthma. Glucocorticoids (GCs) effectively control the disease, but relapses and/or GC-dependence are frequent, leading to immunosuppressant and/or biological therapy use. We examined off-label biological therapy use for relapsing and/or refractory EGPA.
Methods: This retrospective nationwide study included patients with EGPA meeting ACR criteria and/or Chapel Hill Consensus Conference definitions. Treatment efficacy and safety were recorded. Remission was defined as the absence of asthma and/or sinonasal exacerbations and vasculitis manifestations with a prednisone dose ≤7.5 mg/day, and partial response as requiring >7.5 mg of prednisone daily.
Results: Thirty-three patients (22 men, 11 women; median age 50 years) were included. Thirteen (39%) received rituximab (RTX) and 20 (61%) omalizumab (OMA). Previous treatments were: methylprednisolone infusions (54%), oral GCs (100%), IV cyclophosphamide (60%), azathioprine (75%), methotrexate (24%), mycophenolate mofetil (6%), plasmapheresis (6%) and/or co–trimoxazole (33%). RTX was mainly prescribed for pulmonary involvement (92%), sinusitis (69%), peripheral neuropathy (61.5%) and cardiac involvement (46%). OMA was prescribed for pulmonary involvement (90%) and sinusitis (90%). At inclusion, median (range) BVAS for the RTX and OMA groups, respectively, were 4 (0–19) and 2 (0–9). After median follow-up of 20 months, remissions, partial responses and therapeutic failures, respectively, were 38%, 23% and 38% for RTX recipients, and 40%, 20% and 80% for the OMA group. Median BVAS dropped to 0 for both groups. A GC-sparing effect was obtained for both groups but was greater for RTX recipients: median GC dose decreased from the baseline 16 mg/day to 15 at 3 months, 10 at 6 and 12 months and 7.5 at the last follow-up vs. 12.5 mg/day to 11.25 at 3 months, 12 at 6 months, 10 at 12 months and 9 at the last follow-up for the OMA group. Two patients stopped RTX because of severe asthma flares and another because of refractory disease. Eleven patients stopped OMA: 2 because of vasculitis relapses, 2 for severe asthma flares, 4 had refractory disease and 3 achieved remissions. The latter 3 patients then took azathioprine; no relapses occurred after discontinuing OMA. The only minor side effect, nausea, occurred in the RTX group.
Conclusion: The results of this study suggest that RTX and OMA may achieve GC-sparing in relapsing and/or refractory EGPA, but the latter remains frequent.
To cite this abstract in AMA style:Denis L, Samson M, Maurier F, Khouatra C, Germain V, Delbrel X, Bonniaud P, Deroux A, Girszyn N, de Moreuil C, Chauveau D, Gondouin A, Dominique S, Le Guenno G, Bouillet L, Bonnotte B, Kahn JE, Bienvenu B, Godeau B, Le Jeunne C, Puéchal X, Guillevin for the French Vasculitis Study Group L, Terrier B. Off-Label Use of Biological Therapies in Relapsing and/or Refractory Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss) [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/off-label-use-of-biological-therapies-in-relapsing-andor-refractory-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss/. Accessed September 24, 2021.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/off-label-use-of-biological-therapies-in-relapsing-andor-refractory-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss/