Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis disorder which involves multiple organ systems and is characterized by asthma, pulmonary infiltrates, sinusitis, neuropathy, and peripheral eosinophilia. It also has an effect on the heart, skin, kidneys, and gastrointestinal tract. Interlukin-5 (IL-5) is involved in maturation and activation of eosinophil, the production of which is increased in the EGPA. Treatments of EGPA are limited to systemic corticosteroids and immunomodulators. These drugs are associated with significant side effects. Besides this, the response of patients to these drugs may be disappointing. Frequent relapses, the need for long-term medium-to-high-dose glucocorticoid therapy, and failure to achieve remission are not uncommon findings. There is a need for noble agents that could reduce frequent relapses and the dose of systemic glucocorticoids and maintain a sustained remission without significant side effects. Mepolizumab is IL-5 antagonist and may have value in treating patients with EGPA. Therefore, we did a systematic review to evaluate the efficacy and safety of mepolizumab in patients with EGPA.

Highlights

  • Eosinophilic granulomatosis with polyangiitis (EGPA), which was previously recognized as Churg–Strauss syndrome (CSS), was first described in 1951 by Churg and Strauss

  • We reviewed the current evidence for the efficacy and safety of mepolizumab in patients with EGPA

  • A meta-analysis of randomized placebo-controlled trials of mepolizumab in patients with eosinophilic asthma found significantly decreased exacerbation risk compared to placebo (OR, 0.30; 95%CI, 0.13 to 0.67; p=0.004) and a significant improvement in the scores on the Asthma Quality of Life Questionnaire (AQLQ)

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Summary

Introduction

Eosinophilic granulomatosis with polyangiitis (EGPA), which was previously recognized as Churg–Strauss syndrome (CSS), was first described in 1951 by Churg and Strauss. It is a small-vessel necrotizing vasculitis characterized by multisystemic manifestations like asthma, lung infiltrations, extravascular necrotizing granuloma, and hypereosinophilia [1]. Vasculitis of extrapulmonary organs is largely responsible for the morbidity and mortality in patients with EGPA. The prodromal phase is characterized by asthma and/or allergic rhinitis, which usually begins when the individual is in their second to third decade of life. The eosinophilic infiltration phase is characterized by peripheral eosinophilia and eosinophilic tissue infiltration of different organs. The third phase is the vasculitic phase and it is associated with constitutional signs and symptoms like fever, malaise, fatigue, and weight loss

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