摘要
目的探讨IgG4相关性疾病(IgG4-related disease,IgG4-RD)的临床特征及误诊原因。方法对1例多年误诊为炎性假瘤的IgG4-RD临床资料进行回顾分析,并复习相关文献。结果患者因反复出现肝脏占位性病变23年,再发1个月入院。患者分别于23年前、9年前发现肝脏占位性病变在我院行手术切除,术后病理均提示肝脏炎性假瘤。本次因体检再次发现肝脏占位性病变来诊。入院后行腹部CT检查示:肝左叶占位性病变,左肾囊肿;胃镜检查示食管下端及胃小弯肿物。查血清IgG 1590 mg/L,进一步行内镜病理学检查示病变组织有淋巴细胞和浆细胞浸润,综合临床确诊为IgG4-RD。给予糖皮质激素联合免疫抑制剂治疗后,肝、胃占位性病变逐渐缩小,随访1.5年未见新发病变。结论 IgG4-RD常出现多个器官受累或相继受累症状,部分患者因临床症状不典型,极易误诊,及时行血清IgG4及病理学检查可减少误诊。本病对糖皮质激素治疗反应良好,及时规范治疗可控制病情发展甚至治愈。
Objective To explore the clinical characteristics and cause of misdiagnosis of IgG4 related disease ( IgG4-RD). Methods Clinical data of one case of IgG4-related disease misdiagnosed as inflammatory pseudotumor was analyzed retrospectively. Meanwhile, the related literature was reviewed. Results The patient was admitted for hepatic space-occupying lesions several times in the past 23 years, which relapsed 1 month ago. The tumor markers were removed by surgery after a palpable mass was found 23 years ago and 9 years ago respectively, and pathological results showed that the mass was inflammatory pseudotumor. This time the patient visited for another palpable mass in the liver. Later, abdominal CT showed the left hepatie space-occupying lesions and the left kidney cyst and the gastroscope showed masses in the lower esophagus and lesser curvature. In the cause of the disease, it was clearly abnormal that IgG4 level in blood serum was 1590 ng/L and the histopathological examination showed that the pathological tissue was macerated lymphocytes and plasma cells. After a comprehensive analysis of clinical manifestations and various auxiliary examinations, the patient was diagnosed with IgG4 related disease. After treatment with glueocorticoid and immunosuppressive agents ,the liver and stomach space oeeupying lesions began to shrink and no new symptoms appeared during 18 months of follow-up. Conclusion IgG4-RD often involves multiple organs concurrently or sequentially and atypical symptoms of some patients can easily cause misdiagnosis. Comprehensive analysis of the clinical manifestations of the multiple organ damage, serum IgG4 and pathological examination can reduce the misdiagnosis rate of IgG4-RD. The disease responds to glucocorticoid well. Timely and effective treatment can control the development, and even cure the disease.
出处
《临床误诊误治》
2016年第7期31-34,共4页
Clinical Misdiagnosis & Mistherapy