IgG4相关性疾病临床特点分析  

Clinical features of IgG4-related disease

作　　者：曾冉冉 蔡顺天[1] 孙刚[1] 蔡逢春[1] 李闻[1] 黄启阳[1] 张修礼[1] 王淑芳[1] 刘庆森[1] 彭丽华[1] 柴国君[1] 邓小虎[1] 张江林[1] 杨云生[1] 

机构地区：[1]解放军总医院消化病中心,北京100853

出　　处：《中国实用内科杂志》2015年第3期242-245,共4页Chinese Journal of Practical Internal Medicine

摘　　要：目的总结IgG4相关性疾病在中国患者的临床特点、诊断及治疗,提高对该病的认识和临床诊治。方法对解放军总医院2012年1月至2014年9月期间诊断为IgG4相关性疾病的患者的临床资料进行回顾性分析。结果 54例患者被诊断为Ig G4相关性疾病,其中男40例（74.1%）,女14例;平均年龄为（58.6±1.6）岁（35-82岁）;31例（57.4%）出现多器官受累,中位受累器官为2个（2-5个）,受累组织包括胰腺（51.9%）、颌面部腺体（48.1%）、淋巴结（20.4%）、胆管（18.5%）等。94%（51/54）的患者伴有血清IgG4[6.9 g/L（1.4-59.5 g/L）]、IgG、IgE等升高为主的高球蛋白血症,97.4%（37/38）患者糖皮质激素治疗后症状有所缓解或恢复正常。结论 IgG4相关性疾病多见于中老年男性,可累及身体多个部位或组织,以胰腺、颌面部腺体受累最为多见,糖皮质激素治疗有较高疗效。Objective To explore the clinical features,differential diagnosis and management of immunoglobulin 4-related disease（IgG4-RD）. Methods The records of patients diagnosed with IgG4-RD at Chinese PLA General Hospital during Jan 2012 and Sep 2014 were retrospectively analyzed. Results Fifty-four patients（mean age 58.6±1.6 years,40 men and 14 women） were retrieved. Multi-organ involvement was discovered in 31（57.4%） patients,which involved pancreas（51.9%）,lachrymal glands and salivary glands（48.1%）,lymph nodes（20.4%）,bile duct（18.5%）,et al. 51（94.4%） patients were accompanied by the hyperglobulinemia and elevation of serum IgG4 [mean 6.9 g /L（1.4-59.5 g/L）]. While good clinical response to glucocorticoid therapy is observed in the majority of the patients（97.4%）,immunosuppressive or rituximab can be required in case of poor efficacy. Conclusions IgG4-related disease is a rare autoimmune disease which affects mostly middle-aged and elderly men. More than two organs can be involved simultaneously,the commonest sites of involvement were pancreas and salivary gland. Steroid therapy is effective.

关 键 词：IGG4相关性疾病 自身免疫性胰腺炎 免疫球蛋白G 糖皮质激素 

分 类 号：R593.2[医药卫生—内科学]