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Review
. 2018 Jan-Mar;8(1):9-14.
doi: 10.4103/tjo.tjo_12_17.

Immunoglobulin G4-related ophthalmic disease

Wei-Kuang Yu  1   2 , Chieh-Chih Tsai  1   2 , Shu-Ching Kao  1   2 , Catherine Jui-Ling Liu  1   2
Affiliations
Review

Immunoglobulin G4-related ophthalmic disease

Wei-Kuang Yu et al. Taiwan J Ophthalmol. 2018 Jan-Mar.

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized inflammatory disease of unknown etiology. It characterized by distinctive histopathological appearance of dense IgG4-positive lymphoplasmacytic infiltrates, storiform fibrosis, and obliterative phlebitis in one or more organs, simultaneously or subsequently. In cases of ocular adnexal involvement, unique clinicohistopathological features were delineated by recent studies, and IgG4-related ophthalmic disease (IgG4-ROD) is generally recognized as the disease name. A significant proportion of previous labeled idiopathic orbital inflammations and Mikulicz's disease are now consistent with a diagnosis of IgG4-ROD. Increasing studies have accumulated regarding its epidemiology, diagnosis, clinical features, treatment, and the association between lymphoma. In this review, we summarize our present understanding of IgG4-ROD.

Keywords: Idiopathic orbital inflammation; immunoglobulin G4; immunoglobulin G4-related disease; lacrimal gland.

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Conflict of interest statement

The authors declare that there are no conflicts of interests of this paper.

Figures

Figure 1
Figure 1
Clinical manifestation of immunoglobulin G4-related ophthalmic disease. (a) Bilateral lacrimal swelling and mild ptosis in the left eye. (b) Dense immunoglobulin G4-positive lymphoplasmacytic infiltration shown by immunostaining. (c) Computed tomography scan revealed bilateral mass lesion over lacrimal glands
See this image and copyright information in PMC

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