Department of Rehabilitation Sciences, Nagasaki University, 1-7-1 Sakamoto, Nagasaki, 852-8520, Japan,
Rheumatol Int. 2013 Nov;33(11):2931-6. doi: 10.1007/s00296-012-2566-2. Epub 2012 Nov 9.
IgG4-related disease is an emerging disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. In IgG4-related disease, tumor-like lesions develop in multiple organs, such as the lacrimal gland, salivary gland, lung, pancreas, kidney, and lymph nodes. We report here three cases of IgG4-related orbital inflammation that presented as unilateral pseudotumors. The patients all were men, with an age range of 65-75 years. The patients had been pointed out unilateral intra-orbital masses, and histopathological examinations revealed marked accumulation of IgG4-positive plasma cells (IgG4/IgG ratio: 51.1-71.6%) with fibrosis. But storiform fibrosis was seen in only one case, and no obliterative phlebitis was seen. The serum levels of IgG4 were increased to 178-670 mg/dL. The masses had well-defined homogeneous signal intensities, and they were hypo-intense on T1-weighted MR images and iso-intense on T2-weighted MR images. Gadolinium enhanced mass lesions in two cases. All orbital mass lesions responded well to corticosteroid treatment.
IgG4 相关疾病是一种新出现的疾病实体，其特征为血清 IgG4 浓度升高，以及 IgG4 阳性浆细胞浸润导致的肿块或组织肿胀。在 IgG4 相关疾病中，肿瘤样病变可发生于多个器官，如泪腺、唾液腺、肺、胰腺、肾脏和淋巴结。我们在此报告 3 例 IgG4 相关眼眶炎症，表现为单侧假性肿瘤。患者均为男性，年龄 65-75 岁。患者均被指出存在单侧眼眶内肿块，组织病理学检查显示 IgG4 阳性浆细胞明显堆积（IgG4/IgG 比值：51.1-71.6%），伴有纤维化。但仅有 1 例可见席纹状纤维化，无闭塞性静脉炎。血清 IgG4 水平升高至 178-670mg/dL。肿块具有明确的均匀信号强度，T1 加权 MR 图像上呈低信号，T2 加权 MR 图像上呈等信号。2 例病例的肿块增强后呈强化。所有眼眶肿块病变对皮质类固醇治疗反应良好。
