眼眶免疫球蛋白 G4 相关疾病（IgG4-RD）的临床病理特征：病例系列及文献复习。
Clinicopathologic features of orbital immunoglobulin G4-related disease (IgG4-RD): a case series and literature review.
机构信息
National Reporting Centre for Ophthalmic Pathology (NRCOP), Centre For Sight, Ashoka Capitol Building, Banjara Hills, Road No.2, Hyderabad, 500034, India,
出版信息
Graefes Arch Clin Exp Ophthalmol. 2015 May;253(5):803-9. doi: 10.1007/s00417-014-2905-y. Epub 2015 Jan 10.
BACKGROUND
Involvement of orbital structures by immunoglobulin G4-related disease (IgG4-RD) is not uncommon. We conducted this study to evaluate the clinicopathologic features of orbital IgG4-RD.
MATERIAL/METHODS: This was a retrospective, clinicopathologic study. Clinical records, light microscopic features, results of immunostaining with IgG & IgG4 and laboratory findings were reviewed in 16 patients diagnosed with orbital IgG4-RD.
RESULTS
Eleven patients had a bilateral disease, and the lacrimal gland was involved in 14. Dense sclerosis, plasma cell aggregates and dense lymphoplasmacytic infiltrate were seen in all patients. Serum IgG4 titre was elevated in 12 patients. Nine patients responded completely to glucocorticoid treatment. Five patients had a relapse on discontinuation of treatment.
CONCLUSION
Orbital IgG4-RD is a distinct clinicopathologic entity requiring increased awareness and needs to be differentiated from other orbital lymphoproliferative lesions.
背景
眼眶结构受免疫球蛋白 G4 相关疾病（IgG4-RD）累及并不少见。我们开展此项研究旨在评估眼眶 IgG4-RD 的临床病理特征。
材料/方法：这是一项回顾性临床病理研究。我们对 16 例经诊断患有眼眶 IgG4-RD 的患者的临床记录、光镜特征、免疫组化 IgG 和 IgG4 染色结果以及实验室检查结果进行了复习。
结果
11 例患者为双侧病变，14 例患者累及泪腺。所有患者均存在弥漫性硬化、浆细胞聚集和密集的淋巴浆细胞浸润。12 例患者血清 IgG4 浓度升高。9 例患者经糖皮质激素治疗后完全缓解。5 例患者在停止治疗后复发。
结论
眼眶 IgG4-RD 是一种独特的临床病理实体，需要提高认识，并需要与其他眼眶淋巴增生性病变相鉴别。
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