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OBJECTIVES To systematically evaluate, through a Medline search, the role of omalizumab in eosinophilic granulomatosis with polyangiitis (EGPA). METHODS A systematic review was performed with the following inclusion criteria original articles and case reports written in English and reporting an association between omalizumab and EGPA. RESULTS We found 18 papers on EGPA (14 case reports, 3 retrospective cohort studies, 1 prospective cohort study). Omalizumab showed to be effective as corticosteroid-sparing agent in EGPA patients with sev