Bilateral idiopathic orbital inflammation 3 years before systemic Wegener´s granulomatosis in a 7-year-old girl

Bilateral idiopathic orbital inflammation 3 years before systemic Wegener´s granulomatosis in a 7-year-old girl

Juan D Martínez-Gutiérrez1, Enrique Mencía-Gutiérrez1, Esperanza Gutiérrez-Díaz1, José L Rodríguez-Peralto2Departments of 1Ophthalmology and 2Pathology, 12 de Octubre Hospital, Com...

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Autores principales: Juan D Martínez-Gutiérrez, Enrique Mencía-Gutiérrez, Esperanza Gutiérrez-Díaz, José L Rodríguez-Peralto
Formato: article
Lenguaje:EN
Publicado: Dove Medical Press 2008
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Ophthalmology
RE1-994
Acceso en línea:https://doaj.org/article/d4927cde10df43dc8f8520eaaca0c1a4
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spelling oai:doaj.org-article:d4927cde10df43dc8f8520eaaca0c1a42021-12-02T01:31:36ZBilateral idiopathic orbital inflammation 3 years before systemic Wegener´s granulomatosis in a 7-year-old girl1177-54671177-5483https://doaj.org/article/d4927cde10df43dc8f8520eaaca0c1a42008-06-01T00:00:00Zhttp://www.dovepress.com/bilateral-idiopathic-orbital-inflammation-3-years-before-systemic-wege-a1693https://doaj.org/toc/1177-5467https://doaj.org/toc/1177-5483Juan D Martínez-Gutiérrez1, Enrique Mencía-Gutiérrez1, Esperanza Gutiérrez-Díaz1, José L Rodríguez-Peralto2Departments of 1Ophthalmology and 2Pathology, 12 de Octubre Hospital, Complutense University, Madrid, SpainAbstract: Wegener’s granulomatosis (WG) is a necrotizing granulomatous vasculitis characterized by the involvement of the upper or lower airways, lungs, and kidneys, but it can affect almost any organ including the orbit. WG is rare in childhood. This case report describes a 7-year-old girl who presented bilateral idiophatic orbital inflammation and antineutrophil cytoplasmic antibodies-negative titres. Computed tomography scan and magnetic resonance imaging showed enlargement of both lacrimal glands with infiltration. Treatment with corticosteroids achieved remission of the disease. Three years later, she developed a systemic affectation with tracheal stenosis, pulmonary affectation, renal failure, and respiratory tract mucosa inflammation. Lacrimal gland biopsy showed perivascular nonspecific granulomas and ANCA titres remain negative. Treatment with corticosteroids and cyclophosphamide was done. A relapse occurred 2 years later, with complete remission with antitumor necrosis factor-alpha. No other symptoms have appeared after 9 years of follow-up. Early diagnosis and treatment is crucial to increase the survival rate in these patients.Keywords: ANCA, children, idiopathic orbital inflammation, exophthalmos, vasculitis, Wegener’s granulomatosis Juan D Martínez-GutiérrezEnrique Mencía-GutiérrezEsperanza Gutiérrez-DíazJosé L Rodríguez-PeraltoDove Medical PressarticleOphthalmologyRE1-994ENClinical Ophthalmology, Vol 2008, Iss Issue 4, Pp 941-944 (2008)
institution DOAJ
collection DOAJ
language EN
topic Ophthalmology
RE1-994
spellingShingle Ophthalmology
RE1-994
Juan D Martínez-Gutiérrez
Enrique Mencía-Gutiérrez
Esperanza Gutiérrez-Díaz
José L Rodríguez-Peralto
Bilateral idiopathic orbital inflammation 3 years before systemic Wegener´s granulomatosis in a 7-year-old girl
description Juan D Martínez-Gutiérrez1, Enrique Mencía-Gutiérrez1, Esperanza Gutiérrez-Díaz1, José L Rodríguez-Peralto2Departments of 1Ophthalmology and 2Pathology, 12 de Octubre Hospital, Complutense University, Madrid, SpainAbstract: Wegener’s granulomatosis (WG) is a necrotizing granulomatous vasculitis characterized by the involvement of the upper or lower airways, lungs, and kidneys, but it can affect almost any organ including the orbit. WG is rare in childhood. This case report describes a 7-year-old girl who presented bilateral idiophatic orbital inflammation and antineutrophil cytoplasmic antibodies-negative titres. Computed tomography scan and magnetic resonance imaging showed enlargement of both lacrimal glands with infiltration. Treatment with corticosteroids achieved remission of the disease. Three years later, she developed a systemic affectation with tracheal stenosis, pulmonary affectation, renal failure, and respiratory tract mucosa inflammation. Lacrimal gland biopsy showed perivascular nonspecific granulomas and ANCA titres remain negative. Treatment with corticosteroids and cyclophosphamide was done. A relapse occurred 2 years later, with complete remission with antitumor necrosis factor-alpha. No other symptoms have appeared after 9 years of follow-up. Early diagnosis and treatment is crucial to increase the survival rate in these patients.Keywords: ANCA, children, idiopathic orbital inflammation, exophthalmos, vasculitis, Wegener’s granulomatosis
format article
author Juan D Martínez-Gutiérrez
Enrique Mencía-Gutiérrez
Esperanza Gutiérrez-Díaz
José L Rodríguez-Peralto
author_facet Juan D Martínez-Gutiérrez
Enrique Mencía-Gutiérrez
Esperanza Gutiérrez-Díaz
José L Rodríguez-Peralto
author_sort Juan D Martínez-Gutiérrez
title Bilateral idiopathic orbital inflammation 3 years before systemic Wegener´s granulomatosis in a 7-year-old girl
title_short Bilateral idiopathic orbital inflammation 3 years before systemic Wegener´s granulomatosis in a 7-year-old girl
title_full Bilateral idiopathic orbital inflammation 3 years before systemic Wegener´s granulomatosis in a 7-year-old girl
title_fullStr Bilateral idiopathic orbital inflammation 3 years before systemic Wegener´s granulomatosis in a 7-year-old girl
title_full_unstemmed Bilateral idiopathic orbital inflammation 3 years before systemic Wegener´s granulomatosis in a 7-year-old girl
title_sort bilateral idiopathic orbital inflammation 3 years before systemic wegener´s granulomatosis in a 7-year-old girl
publisher Dove Medical Press
publishDate 2008
url https://doaj.org/article/d4927cde10df43dc8f8520eaaca0c1a4
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