Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg–Strauss) is a small-vessel necrotizing vasculitis characterized by blood and tissue eosinophilia and asthma. Glucocorticoids (GCs) represent the cornerstone of treatment. So far, EGPA management has been based on conventional immunosuppressants, but relapses and/or GC-dependence are frequent. More recently, biotherapies targeting B cells and interleukin-5 have been used, but data on large cohorts are lacking. This study aimed to describe therapeutic management and efficacy of conventional immunosuppressants and targeted biotherapies in EGPA patients.

Methods: We started a multicenter European collaborative initiative that included 376 EGPA patients from tertiary referral centers. Remission was defined as the absence of vasculitis manifestations (BVAS=0), asthma and ENT manifestations with a GC dose <7.5 mg/d. Treatment failure was defined as no improvement or worsening and the inability to taper GCs.

Results: After median follow-up of 58.5 (IQR 26.7–101) months, 175 (46.5%) patients were in prolonged remission, 141 (37.5%) had GC-dependent asthma and/or ENT manifestations and 108 (28.7%) had had ³1 vasculitis relapse(s). For remission induction, cyclophosphamide was the most frequently used immunosuppressant (54%), mainly because of cardiac, renal and/or nervous system involvement. Induction conventional immunosuppressants (cyclophosphamide, azathioprine or methotrexate), prescribed based on disease severity, achieved remission rates similar to GCs alone, in terms of overall survival, relapse-free survival and evolution towards 4 different identified profiles. At the first vasculitis relapse, having GC-dependent asthma and/or ENT manifestations and a high GC-dependency threshold were associated with a higher risk of treatment failure (57% vs. 32%, P=0.008). As for induction, conventional immunosuppressants seemed to be similar to GCs alone to achieve remission and lower the relapse risk. Rituximab was prescribed as first-line therapy for 5 patients and for 6 relapsing patients, and achieved remissions in 82%. During follow-up, GC-dependent asthma and/or ENT manifestations were treated with azathioprine (48%), methotrexate (35%), mycophenolate mofetil (16%), cyclophosphamide (10%), cyclosporine (6%) or rituximab (6%), achieving GC-tapering to <7.5 mg/d for 27%, 41%, 42%, 15%, 71% and 29%, respectively. Mepolizumab

Conclusion: Despite their beneficial impact on overall survival of EGPA patients, the efficacies of conventional immunosuppressants proved disappointing, as achieving remissions, preventing vasculitis relapses and controlling GC-dependent asthma and/or ENT manifestations were not improved. Notwithstanding a small number of treated patients, eosinophil-targeted therapies seemed to be promising but their efficacies against vasculitis, asthma and/or ENT manifestations require further investigation.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/efficacy-of-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-treatments-according-to-the-type-of-manifestations-based-on-analysis-of-376-patients/