Long-Term Effectiveness of Benralizumab in Eosinophilic Granulomatosis With Polyangiitis.

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disease characterized by eosinophilic tissue inflammation. Benralizumab, an anti-IL-5 receptor (anti-IL-5R) monoclonal antibody, induces rapid depletion of eosinophils; its longer-term effect in EGPA is unknown. OBJECTIVE: To assess the real-world effectiveness and clinical remission rates of anti-IL-5R therapy in EGPA. METHODS: We performed a retrospective cohort analysis of patients with EGPA, who commenced treatment with benralizumab. Clinical remission, assessed at 1 year and 2 years after the initiation of benralizumab, was defined as an absence of active vasculitis (Birmingham Vasculitis Activity Score of 0) and an oral corticosteroid (OCS) dose of ≤4 mg/d of prednisolone. "Super-responders" were defined as patients in remission and free of any significant relapses (asthma or extrapulmonary) over the preceding 12 months. The corticosteroid-sparing capacity of benralizumab, patient-reported outcome measures, and characteristics associated with clinical remission and super-responder status were also analyzed. RESULTS: A total of 70 patients completed at least 1 year of treatment with benralizumab, of whom 53 completed 2 years. Of 70 patients, 47 (67.1%) met the definition for clinical remission at 1 year, with a similar proportion in remission at 2 years. Excluding asthma-related relapses, 61 of 70 (87.1%) patients were relapse free at 1 year, and of the 53 who completed 2 years, 45 (84.9%) were relapse free. A total of 67.9% of patients no longer needed any OCS for disease control. No significant difference was seen between antineutrophilic cytoplasmic antibody (ANCA)-positive and ANCA-negative subgroups. CONCLUSIONS: In this real-world setting of patients with EGPA, treatment with benralizumab was well tolerated and resulted in corticosteroid-free clinical remission for the majority of patients.

Eosinophilic granulomatosis with polyangiitis: case report and literature review.

Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a multisystem disorder characterised by asthma, blood and tissue eosinophilia and small-vessel vasculitis. Eosinophilic tissue infiltration and extravascular granuloma formation can lead to damage in any organ, but it is classically seen to cause pulmonary infiltrates, sino-nasal disease, peripheral neuropathy, renal and cardiac involvement, and rashes. EGPA is part of the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndromes, with the antibody being detected in ∼30-40% of cases and mostly against myeloperoxidase. Two genetically and clinically distinct phenotypes, defined by the presence or absence of ANCA have been identified. Treatment for EGPA focuses on inducing and maintaining disease remission. To date, oral corticosteroids remain first-line agents whilst second-line treatments include immunosuppressants such as cyclophosphamide, azathioprine, methotrexate, rituximab and mycophenolate mofetil. However, long-term steroid usage results in multiple and well-known adverse health effects and new insights into the pathophysiology of EGPA have allowed for the development of targeted biologic therapies, like the anti-eosinophilic, anti-interleukin-5 monoclonal antibodies.