IgG4相关自身免疫性胰腺炎的临床研究  

Clinical study on IgG4-related autoimmune pancreatitis

作　　者：吴庆军[1] 陈华[1] 林玮[1] 王迁[1] 郑文洁[1] 李梦涛[1] 冷晓梅[1] 徐东[1] 沈敏[1] 费允云[1] 苏金梅[1] 王立[1] 张文[1] 张烜[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,100032

出　　处：《中华风湿病学杂志》2012年第12期798-803,共6页Chinese Journal of Rheumatology

基　　金：基金项目：国家自然科学基金（81172858）

摘　　要：目的探讨IgG4相关自身免疫性胰腺炎（ALP）的临床特征。方法2010年12月至2012年6月间北京协和医院IgG4相关疾病队列研究中纳入50例患者，其中IgG4相关AIP14例，总结其临床、实验室、放射学特点和治疗反应。结果男性9例，女性5例，年龄41～71岁，平均（55-10）岁。梗阻性黄疸（8／14）是胰腺病变的突出表现。胰腺外受累器官包括涎腺10例、淋巴结7例、胆管6例、肺脏6例、肾脏2例、泪腺2例，以及腹膜后组织、肠系膜和前列腺各1例。血清IgG4水平增高[（1．4-35.2）g，L，平均（11±10）g/L]。影像学检查显示胰腺弥漫性“腊肠样”肿大13例，胰体尾肿大1例，伴主胰管弥漫性狭窄4例。12例进行组织病理学检查，显示大量淋巴浆细胞浸润，伴纤维化，IgG4染色阳性。13例应用糖皮质激素或联合免疫抑制剂治疗，取得显著临床和放射学效果。结论IgG4相关AIP具有独特的临床、血清学、影像学和组织病理学改变，糖皮质激素治疗反应良好。Objective To investigate the clinical features of IgG4-related autoimmune pancreatitis （IgG4-related AIP）. Methods A prospective cohort study on IgG4 related disease （IgG4-RD） was carried out in Peking Union Medical College Hospital during December 2010 to June 2012, a total of 50 patients were recruited, including 14 IgG4-related AIP patients. The clinical manifestations, laboratory tests results, radiographic findings, histopatholo-gical lesions and response to treatment were analyzed. Results Fourteen AIP patients accounted for 28% of IgG4-RD, of whom 9 cases were male, 5 females, with the mean age of （55±10） （range 41-71 ） years. Painless obstructive jaundice was the major manifestation of AIP. All patients had extra-pancreatic organ involvement, including salivary glands enlargement （10 cases）, lymph nodes swollen （7 cases）, and bile duct tree （6 cases）, lung （6 cases）, kidney （2 cases）, lacriminal gland （2 cases）, retroperitoneal tissue （1 case）, mesentery （1 case）, and prostate （1 case） involvement. Serum levels of IgG4 subclass in all patients were significantly increased, with mean concentration of （11±10） （range 1.4-35.2） g/L. Radiographic findings showed diffuse （13 cases） or focal （1 case） pancreatic enlargement with irregular narrowing of the pancreatic duct （4 cases）. Histopathological examinations were performed in 12 patients, which revealed massive infiltration with lymphocytes and IgG4 positive plasma cells with remarkable tissue fibrosis. Response to glucocorticosteroid or combined steroid and immunosuppressants were good. Conclusion IgG4-related AIP is a subtype of chronic pancreatitis, which is characterized by specific clinical spectrum, serological markers, radiographic and histopathological features. Glucoeorticosteroid is the first choice, which can significantly improve the clinical and imaging abnormalities.

关 键 词：胰腺炎 自身免疫疾病 IgG4相关疾病 

分 类 号：R576[医药卫生—消化系统]