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中华口腔医学研究杂志(电子版) ›› 2022, Vol. 16 ›› Issue (05) : 322 -327. doi: 10.3877/cma.j.issn.1674-1366.2022.05.010

综述

IgG4相关口腔疾病的研究进展
王妮妮1, 周佳佳1, 张尽美2, 吴亚菲2, 杨靖梅2,()   
  1. 1. 口腔疾病研究国家重点实验室 国家口腔疾病临床医学研究中心,成都 610041
    2. 口腔疾病研究国家重点实验室 国家口腔疾病临床医学研究中心,成都 610041;四川大学华西口腔医院牙周病科,成都 610041
  • 收稿日期:2022-05-16 出版日期:2022-10-01
  • 通信作者: 杨靖梅

Research progress on IgG4-related oral disease

Nini Wang1, Jiajia Zhou1, Jinmei Zhang2, Yafei Wu2, Jingmei Yang2,()   

  1. 1. State Key Laboratory of Oral Diseases & National Clinical Research Center for Oral Diseases, Chengdu 610041, China
    2. State Key Laboratory of Oral Diseases & National Clinical Research Center for Oral Diseases, Chengdu 610041, China; Department of Periodontology, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China
  • Received:2022-05-16 Published:2022-10-01
  • Corresponding author: Jingmei Yang
  • Supported by:
    Science and Technology Planning Project of Sichuan Province(2022NSFSC1521); The Research and Development Foundation of West China Hospital of Stomatology, Sichuan University(RD-02-201905)
引用本文:

王妮妮, 周佳佳, 张尽美, 吴亚菲, 杨靖梅. IgG4相关口腔疾病的研究进展[J]. 中华口腔医学研究杂志(电子版), 2022, 16(05): 322-327.

Nini Wang, Jiajia Zhou, Jinmei Zhang, Yafei Wu, Jingmei Yang. Research progress on IgG4-related oral disease[J]. Chinese Journal of Stomatological Research(Electronic Edition), 2022, 16(05): 322-327.

IgG4相关性疾病(IgG4-RD)是一种非特异性慢性纤维炎症性疾病,累及全身多个器官和系统,需要结合临床表现、血清学检查和组织病理学等诊断。IgG4-RD发病率低,2018年被纳入我国第一批罕见病名录。目前,口腔相关IgG4-RD包括米库利兹病(MD)、Küttner肿瘤等,可累及硬腭、牙龈等黏膜组织。本文就IgG4-RD可能的发病机制、临床特征、诊断标准、治疗及其在口腔相关表现作一综述,以期为临床工作提供帮助。

IgG4-related disease (IgG4-RD) is a non-specific chronic fibroinflammatory disease involving multiple organs and systems, which requires a combination of clinical manifestations, serological examination, and histopathology for diagnosis. IgG4-RD has a low incidence so that it was included in the first batch of rare diseases in China in 2018. At present, oral related IgG4-RD mainly includes Mikulicz′s disease (MD) , Küttner tumor, et al., and it can also involve hard palate, gingiva and other mucosal tissues. This article reviewed the possible pathogenesis, clinical features, diagnostic criteria, treatment and oral related manifestations of IgG4-RD in order to provide clinical guidance.

图1 日本医学会IgG4-RD综合诊断标准(2017年)[22]
表1 IgG4相关性疾病诊治中国专家共识推荐意见[24]
序号 推荐意见 证据级别
1 建议由风湿免疫科主导,多学科联合共同完成1gG4-RD的诊断、评估、治疗和随访  
2 建议依据2011年日本制定的1gG4-RD综合诊断标准及2019年美国风湿病学会(ACR)/欧洲抗风湿病联盟(EULAR)制定的IgG4-RD分类标准进行诊断  
3 血清1gG4升高是IgG4-RD诊断和病情评估的重要指标,但其诊断的特异性不高  
4 影像学检查对1gG4-RD受累器官的诊断和评估有重要的辅助作用,应根据患者受累部位选择适当的检查方法  
5 特征性的病理改变是诊断IgG4-D的重要依据,病理检查对鉴别诊断排除模拟疾病亦至关重要,因此建议有条件者应行组织活检  
6 确诊IgG4-RD的患者应行疾病活动度和严重性的评估  
7 有症状目病情活动的IgG4-RD患者应接受治疗,无症状但重要脏器受累并进展的患者亦需及时治疗  
8 糖皮质激素是治疗IgG4-RD的一线药物 同质队列研究的系统评价
9 免疫抑制剂与糖皮质激素联合使用较单用糖皮质激素更有效控制疾病,减少IgG4-RD患者的复发 单个队列研究(包括低质量随机对照试验,如随访率<80%)
10 难治性或复发性IgG4-RD可选用生物制剂 单个队列研究(包括低质量随机对照试验,如随访率<80%)
11 IgG4-RD复发者的治疗方案需根据患者复发器官、既往用药等情况重新制定 基于经验未经严格论证的专家建议
12 IgG4-RD特殊情况时手术治疗是可选择的治疗方法之一 病例系列研究(包括低质量队列和病例对照研究)
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