A solely ear-involved IgG4-related sclerosing disease with two-years following-up - 26/09/19

Doi : 10.1016/j.anorl.2018.02.002

X. Cheng ^a,^b, Y. Shu ^a,^⁎,^b , B. Chen ^a,^⁎,^b
^a ENT Institute and Otorhinolaryngology Department, Affiliated Eye and ENT Hospital, State Key Laboratory of Medical Neurobiology, Fudan University, Shanghai, China
^b NHC Key Laboratory of Hearing Medicine (Fudan University)/org>, 200031 Shanghai, PR China

^⁎Corresponding authors.

Bienvenue sur EM-consulte, la référence des professionnels de santé.
L’accès au texte intégral de cet article nécessite un abonnement.

pages	3
Iconographies	4
Vidéos	0
Autres	0

Abstract

Introduction

Immunoglobulin G4-related sclerosing disease (IgG4-RSD) is a chronic fibro-inflammatory disease involving systemic multi-organ lesions, such as the salivary and lacrimal glands, lymph nodes and pancreas. The diagnosis of this disease is reliant upon clinical manifestations, laboratory tests, histopathologic results and radiological data. Some studies have found that IgG4-RSD has otological manifestations, whereas there were few studies introducing the diagnosis, therapy and long-term follow-up results of solely otological IgG4-RSD.

Case summary

Here, we report a case of IgG4-RSD involving the ear alone. A female presenting with otalgia and hearing loss underwent surgery, without hormone therapy. The pathological diagnosis was otological IgG4-RSD and no clinical or radiological signs of recurrence were observed at seven and twenty-four months follow-up.

Discussion

This case indicates that IgG4-RSD can invade the ear only, and that surgical therapy without hormone therapy is efficient for solely IgG4-RSD. Pathological results are crucial for diagnosis.

Le texte complet de cet article est disponible en PDF.

Keywords : IgG4-RSD, Solely, Middle ear, Hearing loss