IgG4相关性疾病21例临床分析  

Clinical analysis of 21 cases of IgG4-related diseases

作　　者：吴超[1] 陈建军[1] 王凤英 聂震宇 陈志鸿[2] 周明[1] WU Chao;CHEN Jianjun;WANG Fengying;NIE Zhenyu;CHEN Zhihong;ZHOU Ming(Department of Hematology,the First Affiliated Hospital of Hunan Normal University,Hunan Provincial People＇s Hospital,Hunan,Changsha 410005,China)

机构地区：[1]湖南省人民医院/湖南师范大学第一附属医院血液科,长沙410005 [2]湖南省人民医院/湖南师范大学第一附属医院病理科,长沙410005

出　　处：《疑难病杂志》2018年第9期947-949,共3页Chinese Journal of Difficult and Complicated Cases

摘　　要：目的 分析免疫球蛋白G4相关性疾病(IgG4-RD)的临床特点及诊疗方案。方法 收集2014年4月—2018年4月湖南省人民医院血液科收治的IgG4相关性疾病患者21例,均符合2011年日本科研组公布的诊断标准,对其临床资料进行分析。结果 21例IgG4相关性疾病患者中,男女比例为19:2,主要表现为黄染10例,上腹部疼痛不适4例,发生于恶性肿瘤之后3例,全身乏力合并自身免疫性溶血性贫血1例,进行性消瘦1例,双侧下颌部肿块1例,肝功能异常1例。确诊病种:IgG4相关性自身免疫性胰腺炎13例,IgG4相关性硬化性胆管炎5例,IgG4相关性淋巴组织增生性疾病2例,IgG4相关性肺疾病1例。首诊科室:肝胆科8例,消化科4例,肝病科3例,血液科2例,感染科2例,呼吸科1例,肾内科1例。激素治疗15例,其中14例病情控制后逐渐好转,死亡1例;手术探查或治疗3例;拒绝进一步治疗3例。结论 IgG4相关性疾病常见于中老年男性,临床表现多种多样,可累及全身多个系统,对激素治疗反应较好,但停用激素后可复发。Objective To analyze and investigate the clinical features and diagnosis and treatment of 21 immunoglobulin G4 related diseases. Methods From April 2014 to April 2018, 21 patients with IgG4-related diseases admitted to the Department of Hematology of Hunan Provincial People＇s Hospital were collected. All the patients met the diagnostic criteria published by the Japanese Research Group in 2011, and their clinical data were analyzed. Results Most of the IgG4-related diseases were male,with a male to female ratio of 19：2. The main manifestations were jaundice in 10 cases, 4 cases of epigastric pain discomfort, occurred after malignant tumor in 3 cases,fatigue with autoimmune hemolytic anemia in 1 case,progressive angular in 1 case,bilateral submandibular mass in 1 case, 1 case with abnormal liver function. Confirmed diseases included 13 cases of IgG4-associated autoimmune pancreatitis, 5 cases of IgG4-associated sclerosing cholangitis,2 cases of IgG4-associated lymphoid hyperplasia,and 1 case of IgG4-associated pulmonary disease. First consultation department： 8 cases in hepatobiliary department, 4 cases in digestive department, 3 cases in hepatology department, 2 cases in hematology department, 2 cases in infection department, 1 case in respiratory department and 1 case in nephrology department. 15 cases were treated with hormone therapy,among which 14 cases were gradually improved and 1 case died. Surgical exploration or treatment was performed in 3 cases and no further treatment was performed in 3 cases. Conclusion IgG4-related diseases are common in middle-aged and elderly men. They have a variety of clinical manifestations. It can involve multiple systems of the body, which respond better to hormone therapy but can relapse after stopping hormone therapy.

关 键 词：IGG4相关性疾病 临床特点 病理特点 

分 类 号：R593.2[医药卫生—内科学]