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IGG4-RELATED OPHTHALMIC DISEASE PRESENTING AS CHOROIDAL AND ORBITAL LESIONS

Gange, William S., BA*,†; Holland, Stephen M., MD; De Alba, Felipe, MD

Retinal Cases and Brief Reports: July 2019 - Volume 13 - Issue 3 - p 283–286
doi: 10.1097/ICB.0000000000000580
Case Report
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Purpose: To report a case of IgG4-related ophthalmic disease (IgG4-ROD) which presented as choroidal and orbital lesions.

Methods: Case report.

Results: A 64-year-old man presented with left eye photopsias and a history of IgG4-related perirenal fibrosis. Fundoscopic examination showed multiple bilateral yellow choroidal lesions, and optical coherence tomography showed multiple choroidal lesions. Magnetic resonance imaging of the orbits showed an enhancing lesion present circumferential to the optic nerve, but greater medially, abutting the posterior surface of the left globe. Workup for infectious, autoimmune, and malignant etiologies was negative, and the patient has responded well to treatment with rituximab.

Conclusion: IgG4-related disease is a systemic fibroinflammatory disease, which often presents in another location, as in our patient. In cases of uncertain choroidal and orbital lesions, a thorough workup for other etiologies is indicated, and lymphoma must be ruled out. Steroids are the mainstay of treatment for IgG4-ROD, however, small case series and our patient responded well to rituximab. To our knowledge, this is the first reported case of choroidal and orbital lesions secondary to IgG4-ROD.

IgG4-related ophthalmic disease (IgG4-ROD) is a fibroinflammatory disease which typically presents with lacrimal involvement, leading to dry eye disease. We present the case of a man with biopsy-proven IgG4-related perirenal fibrosis, new onset photopsia, and choroidal and orbital lesions. This is the first reported case of choroidal and orbital lesions secondary to IgG4-ROD.

*Stritch School of Medicine, Loyola University Chicago, Maywood, Illinois; and

Department of Ophthalmology, Loyola University Medical Center, Maywood, Illinois.

Reprint requests: Felipe De Alba, MD, Department of Ophthalmology, Loyola University Medical Center, 2160 South First Avenue, LUH—North Entrance, Suite 2601, Maywood, IL 60153; e-mail: fdealba@lumc.edu

None of the authors has any financial/conflicting interests to disclose.

© 2019 by Ophthalmic Communications Society, Inc.