Orbital Manifestations of Immunoglobulin G4–Related Disease in Bilateral Lacrimal Glands, Optic Nerves, Trigeminal Nerves, and Maxillary Sinuses

Abstract

Immunoglobulin G4 (IgG4)-related disease is characterised by numerous aggregates of IgG4-positive plasma cells in multiple organs. We report two patients who had bilateral proptosis associated with extensive inflammation bilaterally in lacrimal glands, optic nerves, trigeminal nerves, and maxillary sinuses. The patients were treated as idiopathic orbital inflammation syndrome with corticosteroid pulse therapy. As symptoms relapsed upon tapering, a reassessment of immunohistochemical stains of the lacrimal glands confirmed the diagnosis of IgG4-related disease. During 2 years of follow-up, the inflammation regressed spontaneously without any medical treatment in the first patient; however, inflammation in the other patient progressed, and he lost his vision. The extensive orbital involvement, characteristic pathological findings, and slowly progressive clinical course might help practitioners differentiate orbital IgG4-related disease from presumed idiopathic orbital inflammation syndrome.

• Idiopathic orbital inflammatory syndrome
• IgG4-related disease
• Mikulicz disease

Acknowledgements

The first author would like to express her sincere appreciation to Dr. Jon-son Kuo, Department of Medical Research, Buddhist Tzu Chi General Hospital, for his suggestions in editing her dissertation.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

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