摘要
目的总结IgG4相关系统性疾病（IgG4-RSD）的临床特点，提高对该疾病的认识。方法回顾性分析符合lgG4-RSD诊断的8例患者的临床表现、实验室检查、影像学结果、病理表现和治疗及预后情况。结果8例IgG4-RSD患者中，男5例，女3例，中位年龄52．5（36～72）岁；临床首发表现多样化；受累器官包括胰腺（5例）、胆道（3例）、肾脏（6例）、肺部（3例）、后腹膜和腹主动脉（5例）、淋巴结（6例）等；实验室检查中所有患者均存在高球蛋白血症，IgG及IgG4亚型水平升高，部分患者存在贫血（6例）、肾功能异常（5例）、梗阻性黄疸（2例）；不同受累器官均显示相同特点的病理改变即为淋巴浆细胞浸润合并纤维化、IgG4阳性的淋巴浆细胞均〉30个／高倍视野；糖皮质激素治疗反应良好，临床症状改善，IgG、IgG4可明显降低。结论IgG4-RSD临床表现异质；受累器官纷杂；组织病理学特征以病变组织IgGn阳性的浆细胞浸润为其突出特点；糖皮质激素治疗有效。
Objective To explore the clinical features of IgG4-related systemic diseases （IgC,4- RSD）Methods A total of 8 inpatients with IgG4-RSD diagnosed at our hospital during August 2010 to December 2011 were recruited. We analyzed the clinical data, laboratory profiles, radiological and pathologic features and prognostic factors of these patients with IgG4-RSD. Results There were 5 males and 3 females with a mean onset age of 52.5 years. IgG4-related diseases were described in multiple organ systems ： pancreas （ n = 5 ）, biliary tree （ n = 3 ）, kidneys （ n = 6 ）, lungs （ n = 3 ）, aorta and periaortic tissue, retroperitoneum （ n -- 5 ） and lymph nodes （ n = 6 ）. Hyperglobulinemia, elevated serum levels of IgG and IgG4, anemia （ n = 6 ）, renal dysfunctions （ n = 5 ） and obstructive jaundice （ n = 2 ） were common laboratory findings. Lymphoplasmacytic infiltration and fibrosis were common pathologic findings. A diffuse infiltration of plasma cells with over 30 IgCA-positive ceils per high-power field provided compelling evidences of IgG4- related disease. Patients with IgG4-RSD responded well to glucocorticoids. Conclusion With heterogeneous clinical characteristics, IgG4-RSD is found in various organ systems. The prominent histopathologic features of IgG4-RSD include a diffuse infiltration of plasma cells with over 30 IgG4-positive cells per high-power field. And the therapy of glucocorticoids is efficacious.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2012年第42期2988-2991,共4页
National Medical Journal of China
基金
国家科技支撑计划（2001BAll0800）
北京市科技计划（D09050704310901）
