Department of Medicine, Harvard Medical School, Cambridge, Massachusetts, USA.
Surv Ophthalmol. 2012 Jan-Feb;57(1):26-33. doi: 10.1016/j.survophthal.2011.07.004. Epub 2011 Oct 21.
IgG4-related systemic disease (IgG4-RD) is an inflammatory condition of unknown etiology that has been identified as the cause of tumefactive lesions in a number of tissues and organs. The role of the IgG4 remains to be clarified fully, but the histopathologic diagnosis hinges upon the finding of IgG4-bearing plasma cells in addition to characteristic morphologic features, with or without elevated seum IgG4. We present a 56-year-old man with orbital pseudotumor in whom, after 30 years of intractable disease, biopsy showed IgG4-RD involving the lacrimal gland, extraocular muscles, intraconal fat, and trigeminal nerve. Six months after initiating treatment with rituximab, his disease remained dormant, with improvement in his proptosis and normalization of serum IgG4 levels. We review the differential of idiopathic orbital inflammatory disease, including IgG4-RD, and emphasize the need for biopsy for accurate diagnosis and to guide appropriate treatment.
IgG4 相关系统性疾病（IgG4-RD）是一种病因不明的炎症性疾病，已被确定为许多组织和器官肿块样病变的原因。IgG4 的作用仍有待充分阐明，但组织病理学诊断取决于除了特征性形态特征之外，还发现 IgG4 阳性浆细胞，无论血清 IgG4 是否升高。我们介绍了一名 56 岁男性，他患有眼眶假瘤，30 年来病情一直难以控制，活检显示 IgG4-RD 累及泪腺、眼外肌、眶内脂肪和三叉神经。开始使用利妥昔单抗治疗 6 个月后，他的疾病处于休眠状态，眼球突出度改善，血清 IgG4 水平正常化。我们回顾了特发性眼眶炎症性疾病的鉴别诊断，包括 IgG4-RD，并强调了进行活检以进行准确诊断和指导适当治疗的必要性。
