方法：收集整理23例35眼眼眶IgG₄-RD患者的临床病理资料，对其进行组织学和免疫组织化学观察，总结其临床和病理特点。

结果：眼眶IgG₄-RD患者23例35眼，其中男8例9眼，女15例26眼，年龄28～72(平均52.1)岁。19例30眼来源于泪腺，4例5眼来源于眶内其他部位。以泪腺区肿胀或眼球突出就诊。单侧11例，双侧12例。病程1mo～10a，平均27mo。1例1眼6mo后复发。大体：灰白色结节状肿物，泪腺表面有很薄的纤维膜包绕。组织学特点：泪腺腺泡、导管组织严重萎缩甚至消失，被大量密集的淋巴细胞、浆细胞及淋巴滤泡替代，伴有不同程度的纤维化。免疫组织化学染色：23例35眼IgG₄阳性浆细胞均>50个/HPF，IgG₄/IgG阳性浆细胞比值>40%。

结论：眼眶IgG₄-RD主要发生于泪腺组织，通过组织学特点和免疫组织化学IgG₄的表达可明确诊断。IgG₄-RD应早期筛查、预防和治疗。

METHODS: The clinical pathological materials of 23 patients(35 eyes)with orbit IgG₄-RD were collected. They were observed in terms of histology and immunohistochemistry, and its clinical and pathologic characteristics were summarized.

RESULTS: There were 23 patients(35 eyes)with orbit IgG₄-RD(8 male patients, 9 eyes; 15 female patients, 26 eyes), with an average age of 52.1 year-old(from age 28 to 72). 19 patients(30 eyes)occured in lacrimal gland and 4 cases(5 eyes)in other places, and they went to hospital for lacrimal gland cyst or exophthalmos. There were 11 cases in one side and 12 cases in both sides. The disease lasted from 1mo to 10a, averaging 27mo. It recureded in one patient(1 eye)after 1mo. In general inspection: Gray nodular goiter, thin fibrous coat wrapping around the lacrimal gland could be observed. Histologic characteristics: lacrimal gland bubble and catheter group shrinked or even disappeared, substituted by lymphocyte, plasma cells and lymphoid follicle and accompanied with fibrosis. Immunohistochemical staining: IgG₄ positive plasma cells of 23 cases(35 eyes)was >50/HPF, and IgG₄/IgG ratio of positive plasma cells was >40%.

CONCLUSION: Orbit IgG₄-RD mainly occures in lacrimal gland tissue, and expression of IgG₄ can be detected through histologic characteristics and immunohistochemical staining. IgG₄-RD should be screened, prevented and treated in the early phase.