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Journal of Peking University(Health Sciences) ›› 2019, Vol. 51 ›› Issue (1): 1-3. doi: 10.19723/j.issn.1671-167X.2019.01.001

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Clinicopathological characteristics and diagnosis of IgG4-related sialadenitis

Guang-ya YU1,2,△(),Xia HONG1,2,Wei LI1,Yan-yan ZHANG1,Yan GAO3,Yan CHEN3,Zu-yan ZHANG4,Xiao-yan XIE4,Zhan-guo LI5,Yan-ying LIU5,Jia-zeng SU1,Wen-xuan ZHU1,Zhi-peng SUN4   

  1. 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. Center of Stomatology, Peking University Shenzhen Hospital, Shenzhen 518036, Guangdong, China
    3. Department of Oral Pathology, Peking University School and Hospital of Stoma-tology, Beijing 100081, China
    4. Department of Oral Radiology, Peking University School and Hospital of Stoma-tology, Beijing 100081, China
    5. Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing, 100044, China
  • Received:2018-11-26 Online:2019-02-18 Published:2019-02-26
  • Contact: Guang-ya YU E-mail:gyyu@263.net
  • Supported by:
    Supported by the National Natural Science Foundation of China(81470756);Supported by the National Natural Science Foundation of China(81671005);Supported by the National Natural Science Foundation of China(81611540351)

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Abstract:

SUMMARY 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.

Key words: IgG4-related sialadentitis, IgG4-related disease, Salivary gland, Submandibular gland, Parotid gland, Lacrimal gland

CLC Number: 

  • R780.2
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