PDF(Pubmed)
Abstract：
Immunoglobulin G4 (IgG4)-related disease is a clinical entity characterized by elevated serum IgG4 concentrations and infiltration of IgG4-immunopositive plasmacytes in various organs, including ophthalmic lesions. Diagnostic criteria for IgG4-related ophthalmic disease (IgG4-ROD) were established in 2014 and describe the most affected ocular adnexal tissues such as lacrimal glands, trigeminal nerves and extraocular muscles, but do not mention optic neuropathy, the most severe indication of ophthalmic lesions. We reviewed published case reports of optic neuropathy in IgG4-related disease (n = 44), and in many cases, decreased visual acuities recovered well following treatment such as systemic corticosteroids, rituximab, and orbital surgery. However, some patients did not recover, especially when pretreatment visual acuities were as low as light perception or less. Herein, we propose a 2023 revised diagnostic criteria for IgG4-ROD, which include a reminder not to overlook optic neuropathy. The 2014 diagnostic criteria specify mucosa-associated lymphoid tissue (MALT) lymphoma as an important differential diagnosis for the relationship between IgG4-ROD and orbital lymphoma. The 2023 revision directs physicians\' attention toward lymphomas other than MALT lymphoma, considering that the 2014 criteria might have placed too much emphasis on MALT lymphoma.
摘要：
免疫球蛋白G4（IgG4）相关疾病是一种临床实体，其特征是血清IgG4浓度升高和各种器官中IgG4免疫阳性浆细胞浸润，包括眼科病变。2014年建立了IgG4相关眼科疾病诊断标准（IgG4-ROD），并描述了受影响最大的眼部附属器组织，如泪腺。三叉神经和眼外肌，但不要提到视神经病变,眼科病变最严重的指征。我们回顾了已发表的IgG4相关疾病视神经病变的病例报告（n=44），在很多情况下,视力下降后恢复良好，如全身性皮质类固醇治疗，利妥昔单抗,和眼眶手术。然而,一些病人没有康复，特别是当治疗前的视力低至光感或更少时。在这里，我们提出了2023年修订的IgG4-ROD诊断标准，其中包括提醒不要忽视视神经病变。2014年诊断标准将黏膜相关淋巴组织(MALT)淋巴瘤作为IgG4-ROD与眼眶淋巴瘤关系的重要鉴别诊断。2023年修订版将医生的注意力转向MALT淋巴瘤以外的淋巴瘤，考虑到2014年的标准可能过于强调MALT淋巴瘤.
