Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a clinically distinct systemic condition that can involve the orbital tissue. Characterized by a triad of a mass-forming lesion, infiltration by IgG4-positive plasma cells, and elevated serum IgG4 titers in many cases, IgG4-RD has clinicopathologic features that overlap with ocular adnexal lymphomas and orbital inflammatory conditions. Although most cases of orbital IgG4-RD respond well to steroids, it may become necessary to include supplemental immunosuppressant therapy in the management.
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