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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 22 cases of IgG4 related diseases. Methods Retrospective analysis of 22 cases of IgG4-related clinical data. Results Among the 22 cases, the involved organs included 12 cases of pancreas, 4 cases of biliary tract, 5 cases of retroperitoneum, 4 cases of parotid gland in submandibular gland, 4 eyes, 2 cases of kidney, 3 cases of lung, 15 cases of lymph node, 1 case of skin, 1 case of aorta, 9 cases of single organ involvement, 5 cases of 2 organs involvement, 3 cases of 3 or more, 8 cases of onset and 2 to 10 years with an average of 1.7 years with a misdiagnosis rate of 77%. Serum IgG4 (2.3 ~ 22.6 g / L), all of which were found by imaging examination. All cases were treated with hormonal therapy, 17 cases were improved after treatment, and 5 cases were treated with cyclophosphamide or Ai Ruohua Or thalidomide improved after. Conclusion The clinical manifestations of IgG4-related diseases are diverse. For patients with multiple organ-occupying lesions, clinicians should be highly vigilant about the disease, timely diagnosis and avoid unnecessary surgery.