Journal of Neuro-Ophthalmology, March 2014, Volume 34, Issue 1

Bilateral Optic Nerve Involvement in Immunoglobulin G4Related Ophthalmic Disease

To describe a presumptive case of immunoglobulin G4-related ophthalmic disease (IgG4-ROD) with bilateral optic nerve involvement and to review the clinical features of this entity. A 62-year-old man presented with bilateral blurred vision. He had a history of sinus surgery, and a biopsy specimen showed dense infiltration of IgG4-positive plasma cells. His visual acuity was 20/25, right eye, and 20/125, left eye. Serologies demonstrated elevated serum levels of IgG and IgG4, and computed tomography showed masses surrounding both optic nerves at the orbital apices and bilaterally enlarged infraorbital nerves. The patient underwent 2 cycles of intravenous pulse steroid therapy followed by a taper of oral steroids Three months later, vision was 20/20 in each eye and, while the serum level of IgG was within normal limits, the IgG4 level remained elevated. IgG4-ROD may involve the optic nerves resulting in vision loss. Although steroid administration is the primary treatment for this entity, slow tapering is essential to avoid relapse.