Eosinophilic Granulomatosis with Polyangiitis in an 8-year-old Girl Manifesting as Hypereosinophilic Syndrome with Myocarditis, Stroke, and Subsequent Orbital Involvement

Abstract

Hypereosinophilic syndrome (HES) is a heterogeneous group of disorders characterized by peripheral blood eosinophilia of 1.5 × 109/L (1,500/μL) or greater, with evidence of end-organ damage attributable to eosinophilia (e.g., heart, liver or lung) with no other cause for the end-organ damage [1]. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disorder that may affect multiple organ systems (lungs, heart, kidneys, or the nervous system). The disorder is characterized by hypereosinophilia in the blood and tis-sues, inflammation of blood vessels (vasculitis), and the development of inflammatory nodular lesions called granulomatosis [2]. We report a case with a 9-year-old girl presenting with severe hypereosinophilia, ischemic stroke, right-sided hemiparesis and myocarditis treated with methylprednisolone, enoxaparin, rivaroxaban and carvedilol. The patient recovered successfully from myocarditis and stroke but manifested with right-sided orbital involvement as pre-and post-septal orbital cellulitis 10 months later with necrotizing granu-lomatous perivascular chronic infiltration with eosinophilic infiltration treated with methylprednisolone and subsequent mepolizumab with successful remission of orbital involvement, but severe exogenous Cushing’s syndrome and myocardial fibrosis.