Advances in understanding and management of IgG4-related ophthalmic disease
Kenneth Ka Hei Lai, Terence Ang, Wah Cheuk, Angie Lok Ming Kwok, Ming Lin, Yael Lustig, Dinesh Selva, Guy Ben Simon, Yue Xing, Zhi Hui Xu, Hua Sheng Yang, Kelvin Kam Lung Chong, Hunter Kwok Lai Yuen
Abstract
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.