Int J Stomatol ›› 2024, Vol. 51 ›› Issue (2): 193-200.doi: 10.7518/gjkq.2024005

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Diagnosis and treatment progress and clinical characteristics of immunoglobulin G4-related sialadenitis

Yu Zhang1(),Zhiyu Jia2,Huifang Tang1,Zhiyong Zhang1,Wenjing Li1,Songbo Tian1()   

  1. 1.Dept. of Oral Medicine, the Second Hospital of Hebei Medical University, Shijiazhuang 050000, China
    2.Dept. of Oral and Maxillofacial Surgery, the Second Hospital of Hebei Medical University, Shijiazhuang 050000, China
  • Received:2023-02-18 Revised:2023-08-18 Online:2024-03-01 Published:2024-03-11
  • Contact: Songbo Tian E-mail:zhangyuzy99@126.com;tianyx6899@hebmu.edu.cn
  • Supported by:
    Hebei Provincial Natural Science Foundation(H2019206129)

Abstract:

Immunoglobulin G4-related sialadenitis (IgG4-RS) is a chronic autoimmune disease closely associated with IgG4. This disease is an uncommon disorder in the clinical setting, and its etiology is unknown. The major clinical features of IgG4-RS is a painless, persistent enlargement of the salivary gland, especially the submandibular gland. Periphe-ral blood examination show that IgG4 levels are elevated in the serum of most patients, and their lymphoplasmic cells are infiltrated. In the advanced stage of the disease, tissue lesions can present in various organs of the body. Histopathology is the most reliable diagnostic method. Glucocorticoids are recommended as the primary clinical treatment. Biologics can also be used as targeted therapy at this stage. Based on domestic and international literature, we review herein the etiology, clinical manifestations, diagnosis and differential diagnosis, and treatment and prognosis of IgG4-RS to aid the diagnosis, treatment, and subsequent research of the disease.

Key words: immunoglobulin G4, sialadenitis, immunoreaction, diagnosis, treatment

CLC Number: 

  • R781.7+2

TrendMD: 

Tab 1

Differential diagnosis of IgG4-RS"

项目IgG4-RSEHLGSSCOSS
好发人群中老年人,无明显性别差异中年男性中年女性中青年
临床特点易累及下颌下腺,通常双侧1对或1对以上唾液腺持续肿大,易累及泪腺腮腺常见,皮肤粗糙、有瘙痒感,常有过敏症状多个外分泌腺反复肿大,以腮腺最常见,口干症状严重,多有复发性腮腺炎病史多发生于单侧下颌下腺,常在进食后腺体肿大,部分可自行消退,常见导管阻塞症状或导管结石、腺体急性感染病史,无口干症状
实验室检查IgG4水平多升高,自身抗体均为阴性嗜酸性粒细胞增高,IgE水平升高IgG4和IgE 水平常无升高,抗SS-A和抗SS-B抗体和其他自身抗体常阳性IgG及IgG4水平不高
影像学表现B超见低回声不均质团块;CT示腺体浅层密度增高,腺叶不均匀肿胀CT示腺体弥漫性斑片高密度影及结节,皮下脂肪密度增高B超见内部多发小囊状病变,未见腺体浅部低回声带;腮腺造影示末梢导管点、球状扩张B 超见导管内强回声,导管增宽,结石后段导管扩张;X线或CT示高密度结石影
病理组织学大量淋巴浆细胞浸润,部分可见淋巴滤泡形成,腺泡萎缩,编织样纤维化、地图样生发中心;IgG4阳性浆细胞大量浸润同心圆状纤维化,规则生发中心,嗜酸性微脓肿;免疫组织化学结果IgG4阳性淋巴细胞浸润,肌上皮岛形成,无明显纤维组织增生;免疫组织化学结果IgG4阴性导管周围淋巴细胞浸润;免疫组织化学结果IgG4阴性
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