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Journal Français d'Ophtalmologie
Volume 41, n° 6
pages 521-525 (juin 2018)
Doi : 10.1016/j.jfo.2017.10.016
Received : 29 August 2017 ;  accepted : 2 October 2017
Articles originaux

Exophtalmie bilatérale pseudo-tumorale révélant un syndrome hyper-IgG4 orbitaire
Bilateral pseudotumoral proptosis as presenting sign of orbital hyper-IgG4-related disease
 

J. Brour, H. Sassi , W. Koubaa Mahjoub, M. Ouederni, A. Hassairi, A. Chedly Debbiche, M. Chéour
 Hôpital Habib-Thameur, 3, rue A.-Ben-Ayed, 1089 Tunis, Tunisie 

Auteur correspondant. 10, rue de Grenade, Résidence AMAL, Appartement B32, El Manar 1, 2092 Tunis, Tunisie.10, rue de Grenade, Résidence AMAL, Appartement B32, El Manar 1Tunis2092Tunisie
Résumé
Introduction

Le syndrome hyper-IgG4 orbitaire est une cause rare d’exophtalmie bilatérale. Il doit toujours être évoqué, après avoir éliminé le lymphome orbitaire.

Étude de cas

Nous rapportons le cas d’une exophtalmie bilatérale évoluant depuis 4 ans chez un patient de 34 ans. L’IRM orbitaire a montré un processus infiltrant s’étendant à la graisse orbitaire, aux muscles oculomoteurs et aux glandes lacrymales. La biopsie de la glande lacrymale, avec étude en immunohistochimie, a montré un infiltrat lymphoplasmocytaire riche en IgG4 et une fibrose. Le diagnostic de syndrome d’hyper-IgG4 orbitaire a été posé. Le patient a bien répondu à une corticothérapie générale.

Discussion

Le syndrome hyper-IgG4 orbitaire se manifeste le plus souvent par une exophtalmie bilatérale pseudo-tumorale. Les taux d’IgG4 élevés ne sont ni sensibles ni spécifiques. La biopsie avec étude en immunohistochimie est la clé du diagnostic. La corticothérapie générale est le traitement de référence, mais des récurrences peuvent survenir.

The full text of this article is available in PDF format.
Summary
Introduction

Hyper-IgG4 syndrome is a rare cause of bilateral proptosis. It must always be considered after ruling out orbital lymphoma.

Case report

We report a case of progressive bilateral proptosis for 4 years in a 34-year-old man. Orbital MRI showed an infiltrative process extending to the orbital fat, extraocular muscles and lacrimal glands. Lacrimal gland biopsy with immunohistochemical study showed a lymphoplasmocytic infiltrate rich in IgG4 and fibrosis. The diagnosis of orbital hyper-IgG4 syndrome was suggested. The patient responded well to systemic steroid treatment.

Discussion

Orbital hyper-IgG4 syndrome manifests most often as pseudo-tumoral bilateral proptosis. Elevated IgG4 levels are neither sensitive nor specific. Biopsy with immunohistochemical study is the key to diagnosis. Systemic steroid treatment is the gold standard, but recurrences may occur.

The full text of this article is available in PDF format.

Mots clés : Exophtalmie, Syndrome hyper-IgG4, Orbite, Glande lacrymale, Immunohistochimie, Corticothérapie

Keywords : Proptosis, Hyper-IgG4 syndrome, Orbit, Lacrimal gland, Immunohistochemistry, Steroid treatment




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