Advances in understanding and management of IgG4-related ophthalmic disease.

Asia Pac J Ophthalmol (Phila)

Department of Ophthalmology and Visual Sciences, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong, China; Hong Kong Eye Hospital, Hong Kong, China. Electronic address:

Published: October 2024

Immunoglobulin G4-related ophthalmic disease (IgG4-ROD) is an emerging, immune-mediated fibroinflammatory orbital disease, characterized by tumefactive lesions with noticeable IgG4+ plasma cell infiltration and distinctive pathohistological features. This disease is often associated with elevated serum IgG4 concentrations. IgG4-ROD may affect any ophthalmic tissues, particularly the lacrimal gland, extraocular muscles, and trigeminal nerves. Although the exact pathogenic role of IgG4 antibodies remains unclear, B-cell depleting agents have been reported to be an effective treatment. The diverse clinical manifestations of IgG4-ROD complicate diagnosis, and without prompt treatment, visual-threatening complications such as optic neuropathy may arise. Recent advances in understanding and managing IgG4-ROD have revolutionized the diagnosis and treatment of this emerging disease. This review article aims to provide a comprehensive overview of the latest advancements in the field of IgG4-ROD.

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Source
http://dx.doi.org/10.1016/j.apjo.2024.100101DOI Listing

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