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·论著·
IgG4 相关性疾病的临床特征研究
丁航，刘源，张连峰，周琳 * 扫描二维码
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【摘要】 背景 IgG4 相关性疾病（IgG4-RD）是一种少见的慢性炎症伴纤维化疾病，其临床表现缺乏特异性，
易造成漏诊或误诊。目的 分析 IgG4-RD 患者的临床特征。方法 回顾性分析 2015 年 1 月至 2019 年 9 月郑州大学第
一附属医院收治的 106 例 IgG4-RD 患者的临床资料，包括一般资料、实验室检查结果、影像学检查结果、组织病理学
和治疗情况，并通过电话或查询病史资料的方式进行随访，随访时间截至 2020-01-01。结果 106 例 IgG4-RD 患者中，
男 69 例，女 37 例；发病年龄（54.6±13.1）岁。最常见受累器官包括淋巴结（67.0%）、涎腺（37.7%）、胰腺（35.8%）等。
单个器官受累 17 例（16.0%），中位受累器官为 3（2，4）个。女性涎腺、泪腺受累发生率高于男性，胰腺受累发生
率低于男性（P<0.05）。96.1%（99/103）的患者血清 IgG4 ≥ 1.35 g/L，79.6%（82/103）的患者血清 IgG4 ≥ 2.7 g/L。
与血清 IgG4<2.7 g/L 患者相比，血清 IgG4 ≥ 2.7 g/L 患者反应指数（RI）值、嗜酸粒细胞计数、IgG 水平、IgG2 水平、
低补体血症发生率更高以及受累器官数目更多（P<0.05）。胰腺受累组低补体血症发生率高于未受累组，肾脏受累组
低补体血症发生率高于未受累组，涎腺 / 泪腺受累组累及器官数目及嗜酸粒细胞增多症发生率高于未受累组（P<0.05）。
88 例患者接受单用糖皮质激素或激素联合免疫抑制剂治疗，临床缓解率为 97.2%。86 例患者获得随访，其中 32.6%
（28/86）的患者出现复发。结论 IgG4-RD 临床特点在性别以及不同受累器官之间存在差异，血清 IgG4 升高可能代
表更高的疾病活动度，及时使用激素或激素联合免疫抑制剂可有效缓解病情。
【关键词】 IgG4 相关性疾病；临床特点；诊断；激素替代疗法
【中图分类号】 R 593.2 【文献标识码】 A DOI：10.12114/j.issn.1007-9572.2021.01.115
丁航，刘源，张连峰，等 . IgG4 相关性疾病的临床特征研究［J］. 中国全科医学，2022，25（5）：584-588. ［www.
chinagp.net］
DING H，LIU Y，ZHANG L F，et al. Clinical features of IgG4-related disease［J］. Chinese General Practice，2022，25
（5）：584-588.
Clinical Featuresof IgG4-related Disease DING Hang，LIU Yuan，ZHANG Lianfeng，ZHOU Lin *
Department of Gastroenterology，the First Affiliated Hospital of Zhengzhou University，Zhengzhou 450052，China
*
Corresponding author：ZHOU Lin，Chief physician，Professor；E-mail：ZL372@126.com
【Abstract】 Background IgG4-related disease（IgG4-RD）is a rare fibro-inflammatory disease，which is prone
to missed diagnosis or misdiagnosis due to nonspecific clinical manifestations. Objective To analyze the clinical characteristics
of IgG4-related disease（IgG4-RD）. Methods A retrospective design was adopted. Participants were 106 IgG4-RD patients
recruited from the First Affiliated Hospital of Zhengzhou University from January 2015 to September 2019. Their clinical data were
collected，including demographics，results of serological examinations，imaging examinations，histopathological examinations
and treatment status. Data of follow-up conducted by telephone or checking medical history data as of January 1，2020 were also
collected. Results Of the 106 cases，69 were male and 37 were female，with a mean age of onset of（54.6±13.1）years.
The most common organs involved were lymph nodes（67.0%），salivary glands（37.7%），and pancreas（35.8%）. Single
organ involvementoccurred in 17 patients（16.0%）. The median number of involved organs was 3（2，4）. Women had higher
incidence of salivary gland involvement，lacrimal gland involvement as well as lower incidence of pancreatic involvement than men
did（P<0.05）. Serum IgG4≥1.35 g/L was found in 96.1%（99/103）of the patients，and IgG4≥2.7 g/L was found in 79.6%（82/103）
of the patients. Compared with those with IgG4<2.7 g/L，patients with serum IgG4 ≥ 2.7 g/L had higher RI value，serum
eosinophil count，IgG concentration，and IgG2 concentration，and were more prone to hypocomplementemia as well as multiple
organ involvement（P<0.05）. Patients with pancreas and kidney involvement were more likely to develop hypocomplementemia，
whereas eosinophilia and multiple organ involvement were more common in patients with salivary/lacrimal gland involvement
基金项目：2021 年河南省中青年卫生健康科技创新领军人才培养项目（YXKC2021017）
450052 河南省郑州市，郑州大学第一附属医院消化内科
*
通信作者：周琳，主任医师，教授；E-mail：ZL372@126.com
本文数字出版日期：2022-01-13
