title: Orbital Immunoglobulin G4-Related Disease: A Systematic Review.
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.
authors.affiliation: From the *National Reporting Centre for Ophthalmic Pathology (NRCOP) and †Ophthalmic and Facial Plastic Surgery and Ocular Oncology, Centre for Sight, Hyderabad, India.
authors.forename: Kaustubh
authors.initials: K
authors.lastname: Mulay
authors.forename: Santosh G
authors.initials: SG
authors.lastname: Honavar