IgG4相关性疾病346例临床特征分析  

The clinical characteristics of 346 patients with IgG4-related disease

作　　者：张盼盼 赵继志[2] 王木[2] 冯瑞娥[3] 刘小伟[4] 赖雅敏[5] 李雪梅[6] 曾学军[7] 施举红[8] 朱慧娟[9] 薛华丹[10] 张薇[11] 陈华 费允云 彭琳一 曾小峰 张奉春 张文 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院风湿免疫科教育部重点实验室, 100730 [2]中国医学科学院北京协和医学院北京协和医院口腔科, 100730 [3]中国医学科学院北京协和医学院北京协和医院病理科, 100730 [4]中国医学科学院北京协和医学院北京协和医院眼科, 100730 [5]中国医学科学院北京协和医学院北京协和医院消化科, 100730 [6]中国医学科学院北京协和医学院北京协和医院肾内科, 100730 [7]中国医学科学院北京协和医学院北京协和医院普内科, 100730 [8]中国医学科学院北京协和医学院北京协和医院呼吸科, 100730 [9]中国医学科学院北京协和医学院北京协和医院内分泌科, 100730 [10]中国医学科学院北京协和医学院北京协和医院放射科, 100730 [11]中国医学科学院北京协和医学院北京协和医院血液科, 100730

出　　处：《中华内科杂志》2017年第9期644-649,共6页Chinese Journal of Internal Medicine

基　　金：国家自然科学基金（81373190,81571587）;国家重点研发计划（2016YFC0901500）

摘　　要：目的 通过分析346例IgG4相关性疾病（IgG4-RD）患者的临床特征,旨在提高对该病的诊治水平.方法 选2011年1月-2016年1月纳入北京协和医院IgG4-RD前瞻性队列研究且随诊6个月以上的患者.分析患者人口统计学特征、受累器官特点、实验室检查及治疗情况.结果 346例IgG4-RD患者中男230例（66.5％）,女116例（33.5％）,发病年龄（53.8±14.2）岁.最常见的临床表现为淋巴结肿大195例（56.4％）和颌下腺肿大182例（52.6％）.其他器官受累依次为泪腺肿大161例（46.5％）,自身免疫性胰腺炎133例（38.4％）,肺97例（28.0％）,硬化性胆管炎88例（25.4％）,鼻窦81例（23.4％）,腮腺肿大75例（21.7％）,腹膜后纤维化69例（19.9％）,大动脉33例（9.5％）,肾脏24例（6.9％）,皮肤22例（6.4％）,少见受累包括甲状腺、垂体、胃肠道、硬脑膜/硬脊膜、心包、颅内占位、硬化性纵隔炎和睾丸.多数患者为多器官受累,257例（74.3％）患者受累器官≥3个,63例（18.2％）患者2个器官受累,26例（7.5％）患者单个器官受累.IgG4-RD反应指数为13.21±5.70.172例（49.7％）患者有过敏史.285例（94.1％）初治患者血清IgG4升高[8 320（3 764,18 600） mg/L],且与IgG4-RD反应指数呈正相关（r=0.430,P＜0.001）.初始治疗时116例（33.5％）患者单用糖皮质激素,182例（52.6％）患者糖皮质激素联合免疫抑制剂,17例（4.9％）患者单用免疫抑制剂,31例（9.0％）患者未予药物治疗.336例（97.1％）患者病情改善或稳定.结论 IgG4-RD是一种多器官受累的慢性炎症伴纤维硬化性疾病,最常受累器官是淋巴结、颌下腺、泪腺及胰腺.糖皮质激素和免疫抑制剂治疗IgG4-RD有效.Objective To analyze the clinical characteristics of IgG4-related disease （IgG4-RD）so as to improve the understanding of IgG4-RD in China.Methods IgG4-RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016.All patients were followedup for more than 6 months.The demographic characteristics,symptoms,organ involvements,laboratory examinations and treatment efficacy were evaluated and analyzed.Results A total of 346 patients were finally enrolled,including 230 males （66.5%） and 116 fcmales （33.5%）.The mean age of disease onset was （53.8 ± 14.2） years old.The mostly common involved organs were lymph nodes （56.4%） and submandibular glands （52.6%）.Other affected organs and manifestations included：swelling of the lacrimal glands （46.5%）,autoimmune pancreatitis （38.4%）,pulmonary involvement （28.0%）,sclerosing cholangitis （25.4%）,naso-sinusitis （23.4%）,parotid gland swelling （21.7%）,retroperitoneal fibrosis （19.9%）,large arteries involvement （9.5%）,kidney involvement （obstructive nephropathy caused by retroperitoneal fibrosis was excluded） （6.9%）,skin lesions （6.4%）.Rare features consisted of thyroid glands,pituitary glands,gastrointestinal tract,pachymeningitis,pericardium,sclerosing mediastinitis and orchitis.The majority of patients had multi-organ involvement,such as 74.3% patients with 3 and more,18.2% and 7.5% patients with 2 and single organ involvement respectively.The average IgG4-RD responder index （IgG4-RD RI） was 13.21 ±5.70.History of allergy was found in 172 （49.7%） patients.As to the laboratory tests,elevated serum IgG4 levels were confirmed in 285 （94.1%） patients,which was positively correlated with IgG4-RD RI.There were 33.5% patients receiving monotherapy of glucocorticoid,52.6% treated with glucocorticoids combined with immunosuppressive agents,4.9% patients with immunosuppressant only,and 9.0% patients with mild disease not receiving medication.The majority （336,97.

关 键 词：IGG4相关性疾病 队列研究 临床特征 

分 类 号：R593.2[医药卫生—内科学]