Abstract
BackgroundImmunoglobulin G4-related disease is a recently recognized condition with pathologic features that are consistent across a wide range of organ systems. Orbital manifestations of this disease entity typically involve the lacrimal gland and lacrimal duct, extraocular muscles, orbital soft tissue, and sclera. Here, the authors report the first known case of inflammatory choroidal neovascular membrane associated with immunoglobulin G4-related disease and offer suggestions for clinical management of this enigmatic condition.Case presentationA 38-year-old Caucasian man with a history of recurrent tonsillitis presented with blurry vision in his left eye of 1-week duration and was diagnosed as having inflammatory choroidal neovascular membrane. An infectious workup was negative, but his serum immunoglobulin G4 level was elevated at 248 mg/dL (reference 4–86), and a subsequent tonsillectomy for a repeat episode of tonsillitis revealed increased immunoglobulin G4 staining on histopathology, thus confirming the diagnosis of immunoglobulin G4-related disease. The inflammatory choroidal neovascular membrane was treated with intravitreal bevacizumab injections and orally administered prednisone resulting in improved visual acuity and choroidal neovascular membrane regression. He later received rituximab infusions for immunoglobulin G4-related disease.ConclusionsWe report a case of choroidal neovascularization associated with immunoglobulin G4-related disease, a chronic inflammatory condition whose ophthalmic manifestations typically include dacryoadenitis, orbital myositis, or scleritis. This is the first reported instance of inflammatory choroidal neovascular membrane associated with immunoglobulin G4-related disease. Early detection of this disease is important to avoid organ damage and potential complications, so clinicians should maintain an index of suspicion for this condition when inflammatory choroidal neovascular membrane is observed.
Highlights
Immunoglobulin G4-related disease is a recently recognized condition with pathologic features that are consistent across a wide range of organ systems
Inflammatory choroidal neovascular membrane (CNVM) can occur from a disruption in the interface between Bruch’s membrane and the retinal pigment epithelium (RPE), from an angiogenic stimulus mediated by local inflammation, or from a combination of both [2]
During the 4 months, he received two more intravitreal bevacizumab 1.25 mg/0.05 mL injections for recurrence of subretinal fluid associated with inflammatory CNVM in the setting of biopsy-proven immunoglobulin G4 (IgG4)-RD, as well as a 1-month taper of orally administered prednisone starting at 60 mg per day and ending at 5 mg per day, after which the blepharitis resolved
Summary
We report a case of choroidal neovascularization associated with immunoglobulin G4-related disease, a chronic inflammatory condition whose ophthalmic manifestations typically include dacryoadenitis, orbital myositis, or scleritis. This is the first reported instance of inflammatory choroidal neovascular membrane associated with immunoglobulin G4-related disease. Detection of this disease is important to avoid organ damage and potential complications, so clinicians should maintain an index of suspicion for this condition when inflammatory choroidal neovascular membrane is observed
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