摘要
目的总结IgG 4相关疾病(IgG 4-RD)的疾病特征,分析该类疾病早期发病过程中容易被误诊的客观原因。方法回顾性分析7例误诊为恶性肿瘤行手术治疗的IgG 4-RD患者的临床资料、实验室检查、影像学结果、病理表现、治疗和预后情况,并进行随访。结果 7例IgG 4-RD患者中,男5例,女2例,中位年龄为60岁;其中消化系统受累共5例(胰腺3例、胆管2例),甲状腺1例,前列腺1例。实验室检查中,3例患者检测血清IgG 4水平,最高达22.6 g/L,影像学检查均表现为占位性病变;7例患者手术切除组织病理学特征主要为多量淋巴细胞及浆细胞浸润和部分纤维组织增生、免疫组化浆细胞表达IgG 4(+)。部分患者加用糖皮质激素和免疫抑制剂治疗,病情改善。结论 IgG 4-RD临床表现多样,且主要表现为器官或组织占位性病变,易被误诊为恶性肿瘤。组织病理学特征以病变组织IgG 4阳性的浆细胞浸润和纤维化为其突出特点。组织活检病理学检查对辅助诊断和鉴别诊断意义较大。
Objective To summarize the clinical features of IgG4 related disease（ IgG 4-RD） and analyze the causes of misdiagnosis in the early state of this disease. Methods The clinical data,laboratory profiles,radiological,pathologic features,prognostic factors and clinical follow-up of7 patients with IgG 4-RD misdiagnosed as malignant tumors were analyzed. Results There were 5 males and 2 females with a mean onset age of 60 years. IgG 4-RD was described in multiple organ systems,including 3 cases of pancreas,2 cases of biliary ducts,1 case of thyroid gland and 1 case of prostate gland. In the laboratory examination,3 patients detected the serum IgG 4 level,the highest level was up to 22. 6 g/L,and the imaging examination showed the occupying lesion. The pathological features of the 7 patients were the infiltration of multiple lymphocytes and plasma cells and the proliferation of some fibrous tissue,and the expression of IgG 4（ ＋） by immunohistochemical plasma cells. Some patients were treated with corticosteroids and immunosuppressive agents,and their condition improved. Conclusion IgG 4-RD is characterized by multiple clinical manifestations,mainly manifested as occupying lesions of organs or tissues,and is easily misdiagnosed as malignant tumors.Histopathological features were characterized by infiltrating and fibrosis of IgG 4 positive plasma cells.Pathologicalexamination of tissue biopsy is of great significance for auxiliary diagnosis and differential diagnosis.
作者
孙田
魏禺
华冰珠
赵成
SUN Tian;WEI Yu;HUA Bingzhu;ZHAO Cheng(Department of Rheumatology,The Drum Tower Hospital Affiliated to Medical Schoolof Nanjing University,Nanjing,Jiangsu,210008)
出处
《实用临床医药杂志》
CAS
2018年第15期50-52,56,共4页
Journal of Clinical Medicine in Practice
基金
国家自然科学基金(81571581)
关键词
IgG4相关疾病
病理学
恶性肿瘤
误诊
鉴别诊断
lated disease
pathology
malignant tumor
misdiagnosis
differential diagnosis
