Advances in understanding and management of IgG4-related ophthalmic disease

Asia Pac J Ophthalmol (Phila). 2024 Sep-Oct;13(5):100101. doi: 10.1016/j.apjo.2024.100101. Epub 2024 Sep 24.

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.

Keywords: IgG4-related disease; IgG4-related ophthalmic disease; Malignancy.

Publication types

  • Review

MeSH terms

  • Humans
  • Immunoglobulin G* / immunology
  • Immunoglobulin G4-Related Disease* / diagnosis
  • Immunoglobulin G4-Related Disease* / therapy

Substances

  • Immunoglobulin G