Yu G Y, Hong X, Li W, Zhang Y Y, Gao Y, Chen Y, Zhang Z Y, Xie X Y, Li Z G, Liu Y Y, Su J Z, Zhu W X, Sun Z P
Department of Oral & Maxillofacial Surgery, Peking University School and Hospital of Stomatology & National Engineering Laboratory for Digital and Material Technology of Stomatology & Beijing Key Laboratory of Digital Stomatology, Beijing 100081, China.
Center of Stomatology, Peking University Shenzhen Hospital, Shenzhen 518036, Guangdong, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2019 Feb 18;51(1):1-3. doi: 10.19723/j.issn.1671-167X.2019.01.001.
Immunoglobulin G4-related sialadenitis (IgG4-RS) is a newly recognized immune-mediated disease and one of immunoglobulin G4-related diseases (IgG4-RD). Our multidisciplinary research group investigated the clinicopathological characteristics and diagnosis of IgG4-RS during the past 10 years. Clinically, it showed multiple bilateral enlargement of major salivary glands (including sublingual and accessory parotid glands) and lacrimal glands. The comorbid diseases of head and neck region including rhinosinusitis, allergic rhinitis, and lymphadenopathy were commonly seen, which could occur more early than enlargement of major salivary glands. Internal organ involvements, such as autoimmune pancreatitis, sclerosing cholangitis, and interstitial pneumonia could also be seen. Thirty-five (38.5%) patients had the symptom of xerostomia. Saliva flow at rest was lower than normal. Secretory function was reduced more severely in the submandibular glands than in the parotid glands. Serum levels of IgG4 were elevated in almost all the cases and the majority of the patients had increased IgE levels. CT, ultrasonography, and sialography showed their imaging characteristics. Histologically it showed marked lymphoplasmacytic inflammation, large irregular lymphoid follicles with expanded germinal centers, prominent cellular interlobular fibrosis, eosinophil infiltration, and obliterative phlebitis. Their immunohistological examination showed marked IgG-positive and IgG4-positive plasma cell infiltration and high IgG4/IgG ratio. The disease could be divided into three stages according to severity of glandular fibrosis. The serum IgG4 level was higher and the saliva secretion lower as glandular fibrosis increased. IgG4-RS should be differentiated from other diseases with enlargement of major salivary gland and lacrimal gland, such as primary Sjögren syndrome, chronic obstructive submandibular sialadenitis, and eosinophilic hyperplastic lymphogranuloma.
免疫球蛋白G4相关性涎腺炎（IgG4-RS）是一种新认识的免疫介导性疾病，也是免疫球蛋白G4相关性疾病（IgG4-RD）之一。我们的多学科研究团队在过去10年中对IgG4-RS的临床病理特征及诊断进行了研究。临床上，它表现为主要涎腺（包括舌下腺和副腮腺）及泪腺的双侧多发肿大。头颈部的合并症如鼻窦炎、变应性鼻炎和淋巴结病很常见，且可能比主要涎腺肿大出现得更早。还可见到内脏受累，如自身免疫性胰腺炎、硬化性胆管炎和间质性肺炎。35例（38.5%）患者有口干症状。静息唾液流量低于正常。下颌下腺的分泌功能比腮腺更严重地降低。几乎所有病例的血清IgG4水平均升高，且大多数患者的IgE水平升高。CT、超声和涎腺造影显示了其影像学特征。组织学上表现为显著的淋巴细胞浆细胞炎症、生发中心扩大的大型不规则淋巴滤泡、突出的小叶间细胞性纤维化、嗜酸性粒细胞浸润和闭塞性静脉炎。其免疫组织学检查显示显著的IgG阳性和IgG4阳性浆细胞浸润以及高IgG4/IgG比值。根据腺体纤维化的严重程度，该疾病可分为三个阶段。随着腺体纤维化加重，血清IgG4水平升高，唾液分泌减少。IgG4-RS应与其他导致主要涎腺和泪腺肿大的疾病相鉴别，如原发性干燥综合征、慢性阻塞性下颌下涎腺炎和嗜酸性增生性淋巴肉芽肿。
