原发眼眶IgG4相关性疾病的临床分析

Clinical analysis of primary orbital IgG4-related disease

目的总结原发眼眶IgG4相关性疾病的临床症状、体征、诊断、治疗和预后。方法回顾2013年1月至2014年1月在上海长征医院眼科就诊为原发眼眶IgG4相关性疾病患者9例，对其临床症状、眼部体征、实验室及影像学检查、诊断及治疗进行分析。结果该病好发于中老年人，男性多见。临床主要表现为眼球突出伴眶组织肿胀，可伴眼球运动受限。影像学检查多表现泪腺组织病变，可累及眼周边附属器组织。实验室检查结果显示血清IgG4〉1．35g／L。术中见8例瘤体无明显包膜，1例包膜完整。术后组织病理学检查见淋巴细胞、浆细胞浸润及纤维化；IgG4浆细胞浸润，且IgG4细胞超过10个／HPF。随访8～20个月有2例复发。结论眼眶IgG4病易发生于老年人，多侵犯泪腺及其周边组织，血清学及组织病理检查诊断可以明确。

Objective To analyze the clinical manifestations of disease, diagnosis, treatment and prognosis of primary orbital IgG4-related disease. Methods Aretrospective study of 9 cases with primary orbital IG4 related diseases, and the clinical data, including clinical symptoms and signs, laboratory and radiological examination results, and treatment were analyzed. Results The disease usually occurred in elder men. The main clinical manifestations were proptosis and eye lid swelling. Imag- ing often showed enlarged lacrimal gland tissue lesions, partly may involve ocular adnexal surrounding tissue. Lesions from 8 cases had no obvious capsules, but one had. Recurrences were observed in 2 cases during the 8-20 months follow-up time. Conclusions IgG4-related diseases are prone to occur in the elderly, usually involve the lacrimal gland and its surrounding tissue. Serological and histopathological examinations are necessary to make the final diagnosis.