IgG4相关性米库利兹病临床研究  

Clinical research of immunoglobin 4-related Mikulicz＇s disease

作　　者：林玮[1] 陈华[1] 吴庆军[1] 郑文洁[1] 史群[1] 冷晓梅[1] 徐东[1] 沈敏[1] 张文[1] 赵岩[1] 曾小峰[1] 张奉春[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院风湿免疫科、风湿免疫病学教育部重点实验室,100730

出　　处：《中华医学杂志》2013年第13期973-975,共3页National Medical Journal of China

基　　金：国家自然科学基金（81172858）

摘　　要：目的探讨IgG4相关性米库利兹病（MD）的临床特征、治疗及预后。方法自2010年12月北京协和医院开始IgG4相关疾病的前瞻性队列研究，共纳入患者42例，其中18例为MD，分析总结MD患者的临床、实验室特点和治疗反应。结果18例MD患者占同期IgG4相关性疾病42．9％。其中男10例，女8例，平均年龄（48±14）岁。平均随诊8．18个月。全部患者均有唾液腺和泪腺受累，15例合并其他脏器损害，包括自身免疫性胰腺炎7例，硬化性胆管炎4例，淋巴结肿大者6例。10例患者有过敏史。所有患者血清IgG4亚类显著升高。16例进行组织学检查有大量淋巴细胞浸润，淋巴滤泡形成，伴纤维组织增生，IgG4染色阳性。糖皮质激素和免疫抑制剂治疗效果明显。结论MD是一种IgG4相关、以泪腺和唾液腺受累为主的多系统受累的自身免疫病，自身抗体多为阴性，激素治疗反应良好。Objective To explore the clinical features of immunoglobin （IgG4）-related Mikulicz＇s disease （MD）. Methods Since December 2010, a prospective cohort study of IgG4 related disease （IgG4RD） was performed our hospital. A total of 42 IgG4RD patients were recruited along with 18 MD patients. Their clinical, laboratory and histopathological features and response to treatment were analyzed. Results The 18 MD patients accounted for 42. 9% of IgG4RD. There were l0 males and 8 females with a mean age of （48 ± 14） years. The mean follow-up period was 8. 18 months. All of them had an involvement of salivary and lacrimal glands. Fifteen cases suffered other organs＇ damage, including autoimmune pancreatitis （ n = 7 ）, sclerosing cholangitis （ n = 4） and lymph nodes （ n = 6）. And 10 patients complained of a history of allergies. All serum levels of IgG4 subclass significantly increased. Histological examinations in 16 patients revealed massive infiltration of lymphocytes and IgG4 positive plasma cells with obvious tissue fibrosis. Glucoeorticoid and immunosuppressive therapies were effective for MD. Conclusion As an IgG4- related systemic disease involving predominantly lacrimal and salivary glands without autoantibodies, MD is treated optimally with a combination therapy of glucocorticoid and immunosuppressive drugs.

关 键 词：米库利兹病 IGG4相关性疾病 IGG4 

分 类 号：R593[医药卫生—内科学]