第四型免疫球蛋白G相關的眼病變

10.6666/ClinMed.201802_81(2).0018

游偉光；蔡傑智；高淑卿

第四型免疫球蛋白G(IgG4) ； 第四型免疫球蛋白G相關的疾病(IgG4-related disease) ； 不明原因性眼窩發炎症(Idiopathic orbital inflammation)

臨床醫學月刊

81卷2期（2018 / 02 / 23）

93 - 101

繁體中文

第四型免疫球蛋白G相關的疾病(Immunoglobulin G4-related disease, IgG4-related disease)是一種原因不明，但是具有特殊臨床病理特徵的發炎性疾病。IgG4-related disease最具有代表性的變化是在免疫組織染色切片上面表現出第四型免疫球蛋白G染色陽性的淋巴球浸潤，合併席紋狀的纖維化(storiform fibrosis)，以及閉鎖性的靜脈炎。IgG4-related disease可以表現在單一或者多個不同的器官，臨床上可以同時或者先後在不同的器官被診斷。侵犯眼睛及其周圍組織的第四型免疫球蛋白G相關疾病，因文獻顯示其具有特殊的臨床與病理特徵，近來統稱為第四型免疫球蛋白G相關的眼病變(Immunoglobulin G4-related ophthalmic disease, IgG4-related ophthalmic disease)。有部分原本屬於不明原因性眼窩發炎性疾病(idiopathic orbital inflammations)以及Mikulicz氏症(Mikulicz's disease)，現在也被認定是IgG4-related ophthalmic disease。在這篇文章之中，將對於IgG4-related ophthalmic disease的流行病學(epidemiology)，診斷，臨床病徵，治療，做文獻回顧與知識的更新。

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