He Xiaojin, Xing Li, Liu Honggang
Department of Pathology, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.
Department of Pathology, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China. E-mail:
Zhonghua Bing Li Xue Za Zhi. 2014 Dec;43(12):799-804.
To study the clinicopathologic characteristics of IgG4-related disease in the orbital and periorbital tissue.
The clinical manifestations and pathologic features of 17 cases of IgG4-related disease affecting the orbital and periorbital tissue encountered during the period from 2012 to 2013 were studied.
There were 9 male patients and 8 female patients. The age of patients ranged from 11 to 71 years (mean = 48.5 years). The main clinical manifestation was swelling of the eyelids: bilateral in 11 patients and unilateral in 6 patients. The duration of disease ranged from 5 months to 7 years (more than 2 years in 13 cases). Six patients had history of allergic disorders. In addition to orbital/periorbital involvement, the disease also affected salivary gland, lymph node, lung and kidney. The disease relapsed in 9 patients. Amongst the 8 patients treated with steroids, 5 of them achieved complete remission and the remaining 3 patients had partial remission. The IgG4 level of the 17 cases ranged from 1.49 to 14.88 g/L. Histologic examination showed pseudolymphoma pattern in 8 cases, mixed pattern in 8 cases and sclerotic pattern in 1 case. There were various degrees of lymphoplasmacytic infiltrates (with lymphoid follicle formation) and stromal fibrosis. Classical obliterative phlebitis was absent. Tissue eosinophilia was demonstrated in the 17 cases studied. Immunohistochemical study showed the presence of more than 50 IgG4-positive plasma cells per high-power field, with IgG4/IgG plasma cells ratio more than 40%.
There are characteristic clinical manifestations, pathologic features and laboratory findings in orbital and periorbital IgG4-related disease. Thorough understanding is important in arriving at a correct diagnosis.
研究眼眶及眶周组织IgG4相关性疾病的临床病理特征。
对2012年至2013年期间遇到的17例累及眼眶及眶周组织的IgG4相关性疾病的临床表现和病理特征进行研究。
男性患者9例，女性患者8例。患者年龄范围为11至71岁（平均48.5岁）。主要临床表现为眼睑肿胀：11例为双侧，6例为单侧。病程为5个月至7年（13例超过2年）。6例患者有过敏病史。除眼眶/眶周受累外，该疾病还累及唾液腺、淋巴结、肺和肾。9例患者疾病复发。在8例接受类固醇治疗的患者中，5例完全缓解，其余3例部分缓解。17例患者的IgG4水平为1.49至14.88 g/L。组织学检查显示，8例为假淋巴瘤模式，8例为混合模式，1例为硬化模式。有不同程度的淋巴浆细胞浸润（伴有淋巴滤泡形成）和间质纤维化。无典型的闭塞性静脉炎。在所研究的17例病例中均显示有组织嗜酸性粒细胞增多。免疫组织化学研究显示，每个高倍视野有超过50个IgG4阳性浆细胞，IgG4/IgG浆细胞比率超过40%。
眼眶及眶周IgG4相关性疾病有特征性的临床表现、病理特征和实验室检查结果。全面了解对于正确诊断很重要。
