International Review of Ophthalmology ›› 2023, Vol. 47 ›› Issue (2): 163-169.doi: 10.3760/cma.j.issn.1673-5803.2023.02.012

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Pathogenesis and malignant transformation mechanism of IgG4-related ophthalmic diseases

Yan Haihan, Ma Jianmin   

  1. Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital University; Beijing Key Laboratory of Ophthalmology and Visual Science, Beijing 100730, China
  • Received:2022-11-06 Online:2023-04-22 Published:2023-04-22
  • Contact: Ma Jianmin, Email: jmma@sina.com
  • Supported by:
    Beijing Municipal Hospital Management Center Summit plan special fund(DFL20190201); Natural Science Foundation of Beijing (7222025)

Abstract: IgG4-related ophthalmic disease (IgG4-ROD) is an occult-progressive disease characterized by IgG4 and plasma cell infiltration, mattostriate fibrosis, and occlusive phlebitis in tumor-like tissues. At present, the pathogenesis and malignant mechanism of IgG4-ROD remain unclear. After pathogen infection, the pathogen recognition receptor encoded by the innate immune recognition germ line activates B cells, and promotes the activation and class switch to IgG4 of B cells with the assistance of Tfh cells, resulting in the infiltration of IgG4 in tissues. B cells activate cytotoxic T lymphocyte (CTL) cells, which together with CTL cells promote fibrosis. IgG4-ROD can malicious change into mucosa associated lymphoid tissue type (MALT),  immunity and inflammation may be involved in the pathogenesis.(Int Rev Ophthalmol, 2023, 47: 163-169)

Key words: IgG4-related ophthalmic disease, fibrous, mucosa associated lymphoid tissue type lymphoma