48例IgG4相关性疾病患者的临床特征及治疗情况  

Clinical characteristics and treatment of IgG4-related diseases:a report of 48 cases

作　　者：张勇[1] 周合冰[1] Zhang Yong;Zhou Hebing(Department of Hematology,Beijing Luhe Hospital,Capital Medical University,Beijing 101149,China)

机构地区：[1]首都医科大学附属北京潞河医院血液科,101149

出　　处：《中华全科医师杂志》2019年第11期1088-1091,共4页Chinese Journal of General Practitioners

摘　　要：选取2014年1月至2017年1月就诊于首都医科大学附属北京潞河医院的IgG4相关性疾病(IgG4-RD)患者48例,分析其临床特点、组织器官受累情况、实验室检查及治疗情况.结果显示48例患者中位发病年龄为59岁(范围29~78岁).最常见的首发症状是颌下腺肿大10例(20.8%)、腹痛9例(18.8%)、眼睑肿物或水肿9例(18.8%)、黄疸8例(16.7%).最常见的受累器官为颌下腺30例(62.5%),其次为淋巴结26例(54.2%)、泪腺22例(45.8%)、腮腺20例(41.7%)和胰腺15例(31.3%).9例(18.8%)患者行PET/CT检查,SUVmax数值与受累器官数目(r=0.511,P=0.160)及IgG4数值(r=0.385,P=0.306)无明显相关性.组织病理学检查46例(95.8%)可见淋巴细胞、浆细胞增多,26例(54.2%)患者伴纤维组织增生.单药糖皮质激素治疗者23例(47.9%),糖皮质激素联合免疫抑制剂者20例(41.6%),手术治疗者3例(6.3%),病情较轻观察者2例(4.2%).47例(97.9%)患者对初始治疗反应良好.23例糖皮质激素单药治疗组有10例(43.5%)复发,20例糖皮质激素联合免疫抑制剂治疗组有6例(30.0%)复发,复发患者再次给予足量糖皮质激素联合免疫抑制剂治疗仍有效.总之,IgG4-RD是一种多器官受累的慢性炎症伴纤维硬化性改变的疾病,血清IgG4水平升高,病理组织大量IgG4阳性浆细胞浸润,糖皮质激素和免疫抑制剂治疗有效,总体预后良好.Forty eight patients with IgG-related disease(IgG-RD)were treated in Beijing Luhe Hospital,Capital Medical University between January 2014 and January 2017.The clinical features,organ involvement,laboratory findings and treatment of patients were analyzed.The median age of patients was 59(29-78)years old.The most common symptoms were submandibular gland enlargement(20.8%),abdominal pain(18.8%),eyelid mass or edema(18.8%)and jaundice(16.7%).The involved organs mainly were submandibular glands(62.5%),lymph nodes(54.2%),lacrimal glands(45.8%),parotid glands(41.7%)and pancreas(31.3%).PET/CT was performed in 9 cases(18.8%),the SUVmax value was not correlated with the number of affected organs(r=0.511,P=0.160)and IgG4(r=0.385,P=0.306).Histopathology showed increased lymphocytes and plasma cells in 46 cases(95.8%),eosinophilia in 15 cases(31.3%),and fibrous hyperplasia in 26 cases(54.2%).Twenty-three patients(47.9%)were treated with single glucocorticoid,20 patients(41.6%)were treated with glucocorticoid combined immunosuppressants,3 patients(6.3%)received surgical treatment,and 2(4.2%)were observed with watch and wait regimen.Forty(97.9%)patients responded well to the initial treatment.Ten out of 23 cases(43.5%)in corticosteroid monotherapy group relapsed,while 6 out of 20 cases(30.0%)in corticosteroids plus immunosuppressive group relapsed,for whom the full dose of corticosteroid and immunosuppressive agents were still effective.IgG4-RD is a chronic inflammatory and fibrous sclerotic disease with multiple organ involvement,increase serum IgG4 level and tissue infiltration with large number of IgG4+cells.Hormone and immunosuppressive therapy is effective,and the overall prognosis is good.

关 键 词：免疫球蛋白G IGG4相关性疾病 临床特征 治疗结果 

分 类 号：R73[医药卫生—肿瘤]