以眼眶炎性假瘤为突出表现的IgG4相关性疾病的临床研究

摘要

目的 探讨以眼眶炎性假瘤为突出表现的IgG4相关性疾病的临床特征.方法 选取2010年10月至2012年7月于首都医科大学附属北京同仁医院风湿免疫科就诊的以眼眶炎性假瘤为突出表现的IgG4相关性疾病患者11例,回顾性分析其临床表现、实验室检查、影像学特点、组织病理学改变及治疗和转归.结果 11例患者中男性6例,女性5例,平均年龄(51±11)岁,均以眼眶炎性假瘤为突出表现.其中9例患者有≥2个器官受累,眼眶外受累器官包括鼻部(7例)、淋巴结(6例)、唾液腺(4例)、胰腺(2例)、皮肤(1例)、腹膜后(1例)及脾脏(1例).实验室检查显示所有患者血清IgG4水平均升高(≥1.35 g/L).影像学检查显示泪腺弥漫性增大、眼外肌增粗、眶周软组织浸润等.9例患者进行组织病理学检查,提示有大量淋巴细胞、浆细胞浸润,伴纤维化,IgG4染色阳性.11例患者均应用糖皮质激素治疗或联合免疫抑制剂,临床缓解4例,临床改善6例,死亡1例.结论 以眼眶炎性假瘤为突出表现的IgG4相关性疾病临床特征明显,对糖皮质激素治疗反应良好.%Objective To investigate the clinical features of IgG4-related disease with oribital inflammatory pseudotumor.Methods The patients with orbital inflammatory pseudotumor were investigated; 11 caseswere associated with IgG4.The clinical manifestations,laboratory tests results,imaging characteristics,histopathology and response to treatment were analyzed.Results In 11 patients,6 cases were male and 5 cases were female.The mean age was(51 ± 11)years.Nine patients had 2 or more organs involvement,including salivary gland (4 cases),the pancreas(2 cases),nose (7 cases),lymph nodes (6 cases),skin (1 case),retroperitoneal tissue (1 case) and spleen (1 case).The serum level of IgG4 of patients was increased (≥ 1.35 g/L).Radiographic findings showed diffused enlargement of lacrimal gland,extraocular muscule hypertrophy and infiltration of orbital soft tissue.Nine cases were histopathologically checked and showed a lot of lymph cells and plasma cells infiltration with fibrosis; IgG4 staining was positive.Response to glucocorticosteroid or combined steroid with immunosuppressants were good except that 1 case died.Conclusions Clinical features of the IgG4-related disease in the orbital inflammatory pseudotumor for outstanding performance is significant.There is a good response to corticosteroid therapy.