眼附属器 IgG4相关性疾病的临床病理分析  

Clinicopathologic characteristics of IgG4-related disease in orbital and periorbital tissue

作　　者：何小金[1] 邢莉[1] 刘红刚[1] 

机构地区：[1]首都医科大学附属北京同仁医院病理科头颈部分子病理诊断北京市重点实验室,100730

出　　处：《中华病理学杂志》2014年第12期799-804,共6页Chinese Journal of Pathology

基　　金：国家自然科学基金（81070769）;北京市卫生系统高层次卫生技术人才培养计划（2009-3-31）

摘　　要：目的：探讨眼附属器IgG4相关性疾病（ IgG4－RD ）的临床病理学特征。方法收集2012至2013年眼附属器淋巴组织增生性疾病的病例103例，对其进行组织学观察、IgG和IgG4免疫组织化学EnVision法染色及血清学IgG和IgG4浓度检测，选取符合IgG4－RD诊断标准的病例17例，分析总结其临床和病理改变特点。结果（1）17例眼附属器IgG4－RD，男性9例，女性8例。年龄11～71岁，平均年龄48．5岁。17例均以眼睑肿胀或眼球突出就诊，11例可见双侧累及，6例为单侧累及。病程从5个月到7年，其中13例病程＞2年。6例既往有过敏性疾病（药物食物过敏）史。眼外常累及的部位有淋巴结、涎腺、肺脏和肾脏等。9例术后出现复发。5例口服激素有效，3例为部分有效。（2）血清学检查：17例IgG4浓度均＞1．35 g／L（1．49～14．88 g／L）。（3）组织学上，假性淋巴瘤样模式8例，混合型模式8例，硬化型1例。14例病变位于泪腺，3例病变累及眶内脂肪结缔组织，未见发生于结膜及眼睑的病例。17例中均可见到不同程度淋巴细胞和浆细胞浸润伴淋巴滤泡形成，程度不一的组织纤维化及嗜酸性粒细胞浸润，但未见典型的闭塞性静脉炎。（4）免疫组织化学染色：17例IgG4阳性浆细胞绝对值均＞50个／HPF， IgG4／IgG阳性浆细胞比值＞40％。结论眼附属器IgG4－RD在临床表现、实验室检查和组织学表现上都具有一定的临床病理学特征，应注意与该部位近似病变的鉴别诊断。Objective To study the clinicopathologic characteristics of IgG4-related disease in the orbital and periorbital tissue.Methods The clinical manifestations and pathologic features of 17 cases of IgG4-related disease affecting the orbital and periorbital tissue encountered during the period from 2012 to 2013 were studied.Results There were 9 male patients and 8 female patients.The age of patients ranged from 11 to 71 years （ mean =48.5 years ） .The main clinical manifestation was swelling of the eyelids：bilateral in 11 patients and unilateral in 6 patients.The duration of disease ranged from 5 months to 7 years （more than 2 years in 13 cases） .Six patients had history of allergic disorders.In addition to orbital/periorbital involvement, the disease also affected salivary gland, lymph node, lung and kidney.The disease relapsed in 9 patients.Amongst the 8 patients treated with steroids, 5 of them achieved complete remission and the remaining 3 patients had partial remission.The IgG4 level of the 17 cases ranged from 1.49 to 14.88 g/L.Histologic examination showed pseudolymphoma pattern in 8 cases, mixed pattern in 8 cases and sclerotic pattern in 1 case.There were various degrees of lymphoplasmacytic infiltrates （ with lymphoid follicle formation） and stromal fibrosis.Classical obliterative phlebitis was absent.Tissue eosinophilia was demonstrated in the 17 cases studied.Immunohistochemical study showed the presence of more than 50 IgG4-positive plasma cells per high-power field, with IgG4/IgG plasma cells ratio more than 40%. Conclusion There are characteristic clinical manifestations, pathologic features and laboratory findings in orbital and periorbital IgG4-related disease.Thorough understanding is important in arriving at a correct diagnosis.

关 键 词：眼疾病 免疫球蛋白G 淋巴组织增殖性疾病 

分 类 号：R77[医药卫生—眼科]