IgG4相关性疾病临床特征分析

Clinical analysis of patients with IgG4-related disease

目的针对IgG4相关性疾病（IgG4-RD）的临床特点、影像学特征、组织病理学表现及预后进行分析，为IgG4-RD的诊断及治疗提供依据。方法回顾性收集2011～2015年在四川大学华西医院诊治的48例诊断为IgG4相关性疾病患者临床资料，统计分析该病患者症状，血清IgG和IgG4、肿瘤血清学标志物及自身抗体表达，总结该病患者CT、MRI、超声等影像学特点，随访疾病预后。结果48例IgG4-RD平均年龄（50±16）岁，其中男女比例为1．5：1，血清IgG4水平在0．05～80．5g／L之间。淋巴结受累患者最多，14例患者伴有多部位受累。共有47例患者进行组织病理学检查，主要表现为淋巴浆细胞浸润及纤维化形成，IgG4＋细胞〉10个高倍视野。38例患者进行糖皮质激素联合免疫抑制剂治疗，激素减量后，2例患者疾病复发。结论IgG4-RD是多器官受累的自身免疫病，受累脏器IgG4阳性浆细胞浸润是其主要特点，及时应用糖皮质激素及免疫抑制剂可有效缓解病情。

Objective To respectively summarize clinical, radiographic, and histopathological features of patients with IgG4 related disease （IgG4-RD） and treatments for the diseases. Methods 48 patients with IgG4-RD hospitalized in West China Hospital of Sichuan University from 2011 to 2015 were enrolled into this respective study. Symptoms and laboratory tests, such as IgG, IgG4 and antibodies, of patients were reviewed. The characteristics of radiologic images and ultrasound were summarized. Following up of patients were also recorded. Results Among all the subjects,the ratio of male to female was 1.5：1, while the mean age was （50 ± 16） years old. The serum level of IgG4 ranged from 0.05 to 80.5 g/L. The most frequently involved organ was lymph gland. 14 patients presented more than two organ impaired. Histopathological findings showed infiltration of various cells in tissues,including lymphocytes, IgG＋ plasma cells, IgG4 ＋ plasma cells and diffuse fibrosis. The IgG4 ＋ plasma ceils were over 10/high power fields（HPF）. Good responses to glucocorticoid in combination with immunosuppressants were observed, except 2 patients with relapsing. Conclusion IgG4-RD is an autoimmune disease affecting multiple organs,and increased IgG4 positive plasma cells is a characteristic feature of the disease. This disease can be effectively alleviated by proper use of glucocorticoid.