[关键词]
[摘要]
近年来，免疫球蛋白G4(IgG4)相关性疾病作为一种新的自身免疫性疾病，受到国内外医学界的的广泛关注。IgG4相关性疾病以IgG4+浆细胞浸润和血清IgG4浓度升高为特征，同时伴有因全身多器官的纤维化而发生肿大、结节性或增生性病变的自身免疫性疾病。当累及眼眶时称为IgG4相关性眼眶病。目前提出了IgG4相关性眼眶病的相对完善的诊断标准，然而治疗方面还没有公认有效的手段，目前首选激素治疗。本文就IgG4相关性眼眶病的临床特征、诊断和治疗的研究进展进行综述。
[Key word]
[Abstract]
Immunoglobulin G4-related disease(IgG4-related disease)has received lots of attention in medical community as a recently recognized fibro-inflammatory condition. It is characterized by infiltration of IgG4-immunopositive plasmacytes and concentration of elevated serum IgG4. IgG4-related disease shows organ enlargement or nodular/hyperplastic lesions in various organs including the pancreas, hepatobiliary tract and orbit, which is called IgG4-related orbital disease. The diagnostic criteria for IgG4-related disease and IgG4-related orbital disease has recently been established, which is based on clinical, imaging and histopathologic features of the orbital lesions. Besides, attention should be drawn to the differentiation from other diseases. The treatment is empirical including corticosteroids, immunosuppressive drugs, radiotherapy, and rituximab. This article reviews clinical progression of IgG4-related orbital disease.
[中图分类号]
[基金项目]
国家高技术研究发展计划(863计划)(No.2015AA020311)
