Clinicopathological characteristics and diagnosis of IgG4related sialadenitis.
10.19723/j.issn.1671-167X.2019.01.001
- Author:
Guang Yan YU
1
;
Xia HONG
1
;
Wei LI
1
;
Yan Yan ZHANG
1
;
Yan GAO
2
;
Yan CHEN
2
;
Zu Yan ZHANG
3
;
Xiao Yan XIE
3
;
Zhan Guo LI
4
;
Yan Ying LIU
4
;
Jia Zeng SU
1
;
Wen Xuan ZHU
1
;
Zhi Peng SUN
3
Author Information
1. 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.
2. Department of Oral Pathology, Peking University School and Hospital of Stomatology, Beijing 100081, China.
3. Department of Oral Radiology, Peking University School and Hospital of Stomatology, Beijing 100081, China.
4. Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, 100044, China.
- Publication Type:Journal Article
- MeSH:
Autoimmune Diseases;
Humans;
Immunoglobulin G;
Sialadenitis;
Sjogren's Syndrome;
Submandibular Gland
- From:
Journal of Peking University(Health Sciences)
2019;51(1):1-3
- CountryChina
- Language:Chinese
-
Abstract:
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.