IgG4相关性疾病的临床诊断学特征

Diagnostic features of IgG4-related disease

目的探讨IgG4相关性疾病(IgG4-RD)的临床诊断学特征。方法回顾性分析2015年1月至2018年6月泰安市中心医院经病理科确诊的3例IgG4-RD患者的临床资料,观察IgG4-RD的病理形态学特点及免疫表型。结果患者中男性2例,女性1例,年龄76～80岁; 3例患者均血清IgG4升高; CT检查可见相应部位或器官弥漫性或局限性肿大;病灶标本光镜下均可见弥漫或片状淋巴细胞及IgG4+浆细胞浸润;浸润细胞周围有特征性的纤维化,典型呈"螺旋环"状,伴有腮腺、肾小管等的萎缩。结论 IgG4-RD是一种少见的慢性自身免疫性疾病,以高血清IgG4水平及组织中大量IgG4+的浆细胞浸润、伴有显著纤维化为特征;临床、影像学及组织学表现均易与肿瘤等疾病相混淆,掌握其临床特征并结合免疫组化方能正确诊断。

Objective To explore the clinical diagnostic features of IgG4-related disease （IgG4-RD）. Methods The clinical data of 3 cases of IgG4-RD diseases confirmed by Pathology Department of Tai′an Central Hospital from January 2015 to June 2018 were analyzed retrospectively, and the pathomorphological features and immunophenotype of IgG4-RD were observed and their clinical diagnostic features were analyzed. Results Among the 3 patients, 2 cases were males and 1 case was female. Their age at the time of operation ranged from 76 to 80 years. All the patients with elevated plasma IgG4. Diffuse or flaky lymphocytes and IgG4 ＋ plasma cell infiltration were observed under light microscope. Characteristic fibrosis was visible around the infiltrating cells. Typical fibrosis can be a spiral ring structure. It was accompanied by atrophy of normal tissues and organs such as the parotid gland and renal tubules. Conclusions IgG4-RD is a rare chronic autoimmune disease, which is characterized by a high serum IgG4 level and a large number of IgG4 positive plasma cell infiltration and significant fibrosis in the tissue. It is easily confused with tumor and other diseases in clinic, imaging and histology. Thorough understanding of the associated clinical features and immunohistochemic results is helpful in achieving the correct diagnosis.