Jpn J Ophthalmol. 2013 Nov;57(6):573-9. doi: 10.1007/s10384-013-0266-4. Epub 2013 Aug 7.
Immunoglobulin (Ig)G4-related ophthalmic disease belongs to a category of ocular adnexal lymphoproliferative disorders, the most frequent group of orbital tumors and simulating lesions. The aim of this study was to elucidate the number of IgG4-related diseases of orbital lymphoproliferative disorders and correlate ages and sex of such patients from 18 centers in Japan.
One thousand and fourteen patients with orbital lymphoproliferative disorders were enrolled in this study. All had pathologically diagnosed lymphoproliferative disorders with surgical samples of ocular adnexal tissue. Patients with conjunctival lesions and intraocular lymphoma were excluded.
Of the 1,014 cases of orbital lymphoproliferative disorders 404 (39.8 %) had extranodal mucosa-associated lymphoid tissue (MALT) lymphoma, 156 (15.4 %) had other malignant lymphomas, 191 (18.8 %) had non-IgG4 orbital inflammation, 219 (21.6 %) had IgG4-related orbital inflammation, and 44 (4.3 %) had IgG4-positive MALT lymphoma. Median age of the IgG4-related orbital inflammation group was 62 years, which is significantly lower than that of the MALT lymphoma group (median 66 years) and higher than the non-IgG4 orbital inflammation group (median 57 years). The male/female ratio was 105/114 in the IgG4-related orbital inflammation group.
Nearly a quarter of orbital lymphoproliferative disorders in Japan are related to IgG4.
免疫球蛋白(IgG4)相关眼病属于眼部附属器淋巴组织增生性疾病,是眼眶肿瘤和模拟病变中最常见的一组。本研究旨在阐明日本 18 个中心的 IgG4 相关疾病在眼眶淋巴组织增生性疾病中的数量,并对这些患者的年龄和性别进行相关性分析。
本研究纳入了 1014 例眼眶淋巴组织增生性疾病患者。所有患者均经病理诊断为淋巴组织增生性疾病,并进行了眼部附属器组织的手术样本检查。排除了患有结膜病变和眼内淋巴瘤的患者。
在 1014 例眼眶淋巴组织增生性疾病患者中,404 例(39.8%)患有结外黏膜相关淋巴组织(MALT)淋巴瘤,156 例(15.4%)患有其他恶性淋巴瘤,191 例(18.8%)患有非 IgG4 眼眶炎症,219 例(21.6%)患有 IgG4 相关眼眶炎症,44 例(4.3%)患有 IgG4 阳性 MALT 淋巴瘤。IgG4 相关眼眶炎症组的中位年龄为 62 岁,明显低于 MALT 淋巴瘤组(中位年龄 66 岁),高于非 IgG4 眼眶炎症组(中位年龄 57 岁)。IgG4 相关眼眶炎症组的男女比例为 105:114。
日本近四分之一的眼眶淋巴组织增生性疾病与 IgG4 相关。