Please use this identifier to cite or link to this item: 10.15388/Amed.2023.30.1.5
Title: Eosinophilic Granulomatosis with Polyangiitis in an 8-year-old Girl Manifesting as Hypereosinophilic Syndrome with Myocarditis, Stroke, and Subsequent Orbital Involvement
Authors: Panina, Aleksandra
Ligere, Elīna
Aleksejeva, Elīna
Davidsone, Zane
Cebure, Elizabete
Erdmane, Irina
Department of Paediatrics
Keywords: Churg–Strauss syn-drome;eosinophilic myocarditis;orbital involvement;paediatric hypereosinophilic syndrome;stroke;3.2 Clinical medicine;1.1. Scientific article indexed in Web of Science and/or Scopus database;General Medicine
Issue Date: 24-Jan-2023
Citation: Panina , A , Ligere , E , Aleksejeva , E , Davidsone , Z , Cebure , E & Erdmane , I 2023 , ' Eosinophilic Granulomatosis with Polyangiitis in an 8-year-old Girl Manifesting as Hypereosinophilic Syndrome with Myocarditis, Stroke, and Subsequent Orbital Involvement ' , Acta medica Lituanica , vol. 30 , no. 1 , pp. 45-52 . https://doi.org/10.15388/Amed.2023.30.1.5
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.
Description: Copyright © 2023 A. Panina Aleksandra, Ligere Elīna, Aleksejeva Elīna, Davidsone Zane, Cebure Elizabete, Erdmane Irina. Published by Vilnius University Press.
DOI: 10.15388/Amed.2023.30.1.5
ISSN: 1392-0138
Appears in Collections:Research outputs from Pure / Zinātniskās darbības rezultāti no ZDIS Pure

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