IgG4相关性疾病22例临床分析

Clinical analysis of IgG 4-related disease

目的分析22例IgG4相关性疾病的临床特点。方法回顾分析22例IgG4相关性疾病的临床资料。结果 22例中,受累器官包括胰腺12例,胆道4例,腹膜后5例,颌下腺腮腺4例,眼4例,肾2例,肺3例,淋巴结15例,皮肤1例,鼻窦1例,主动脉1例;单个器官受累9例,2个器官受累5例,3个及以上8例;从发病到确诊时间:2周~10年,平均1.7年,误诊率77%;所有病例血清IgG4水平均有不同程度升高(2.3~22.6 g/L),影像学检查均发现器官占位;所有病例均行激素治疗,17例治疗后明显好转,5例加用环磷酰胺或爱若华或沙利度胺后好转。结论 IgG4相关性疾病临床表现多样,对多器官占位病变者,临床医生应高度警惕该病,及时诊断,避免不必要的手术。

Objective To analyze the clinical features of IgG4-related disease.Methods The clinical data of 22 patients with IgG4-related disease were retrospectively analyzed.Results The involved organs or tissues including the pancreas in 12 cases,bile duct in 4 cases,retroperitoneal space in 5 cases,salivary glands in 4 cases,eyes in 4 cases,renal in 2 cases,lymph nodes in 15 cases,lung in 3 cases,skin in 1 case,paranasal sinus in 1 case and aorta in 1 case;single organ involved in 9 cases,two organs involved in 5 cases,three or more organs involved in 8 cases.Confirmed time was from 2 weeks to 10 years,whith an average of 1.7 years,the misdiagnosed rate was 77%; all of the cases showed increased levels of serum IgG4,and imaging revealed organ occupying lesions.All of the patients were treated with corticosteroid,17 cases were obviously improved after treatiment and 5 cases were improved after treated with cyclophosphamide in addition.Conclusions IgG4-related disease shows complicated and varied clinical manifestations,so clinicians should keep vigilance against the patients with multiple organ mass,and avoid unnecessary surgery.