An Overview of Immunoglobulin G4-related Ophthalmic Diseases Accompanied by a Case Report

Abstract

Immunoglobulin G4-related diseases (IgG4-RDs) are immune-mediated, fibroinflammatory conditions that are characterised by affected organ enlargement, lymphocyte, plasma cell infiltration (determined with IgG4-positive plasma cells) and serum IgG4 le vel eleva- tion. When the disease affects ocular adnexal tissues (such as the lacrimal gland, extraocular muscles, trigeminal nerve branch es and orbital fat), it is called IgG4-related ophthalmic disease (IgG4-ROD). The diagnosis of IgG4-ROD is made by physical examination, biochemical find- ings and histopathological evaluation. It is characterized by elevated serum IgG4 levels and distinctive histopathological feat ures, including IgG4+ plasma cell infiltration, the presence of fibrosis in the storiform pattern and the presence of a dense lymphoplasmacyticinflammatory infiltrate, including eosinophils. It is rare in the ocular region and cause diagnostic confusion. There is a high probabilityof misdiagnosis when the disease is not recognized. Here, a rare case of eye involvement is presented and IgG4-RDs are discussed with the literature.