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Peer-reviewedCC BY-NC-ND 4.0Medical Science Monitor: International Medical Journal of Experimental and Clinical Research

Clinical Analysis and Diagnostic Challenges of Immunoglobulin G4-Related Disease Involving the Salivary Gland: A Single Center Retrospective Analysis of 24 Cases

  • Tonghui Ding1
  • Hongyuan Chen2
  • Yabing Chen3
  • Yuting Xu4
  • Jiaming Mao5
  • Jianjie Song1
  • Chengjing Zhu1
  • Wei Wang1
5 affiliations
  1. 1Department of Otorhinolaryngology Head and Neck Surgery, Changhai Hospital Affiliated with The Second Military Medical University of PLA, Shanghai, PR China
  2. 2Department of Pathology, Changhai Hospital Affiliated with The Second Military Medical University of PLA, Shanghai, PR China
  3. 3Department of Rheumatology and Immunology, Changhai Hospital Affiliated with The Second Military Medical University of PLA, Shanghai, PR China
  4. 4Department of Oral and Maxillofacial Surgery, Changhai Hospital Affiliated with The Second Military Medical University of PLA, Shanghai, PR China
  5. 5Department of Medical Imaging, Changhai Hospital Affiliated with The Second Military Medical University of PLA, Shanghai, PR China
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Abstract

Background: Immunoglobulin G4–related disease (IgG4-RD) is a rare and complex autoimmune disorder, and there is a lack of awareness in surgical settings for patients with salivary gland involvement. This study summarized the clinical characteristics of IgG4-RD involving the salivary gland, aiming to improve clinical physicians’ understanding, diagnosis, and treatment of IgG4-RD.

Material/Methods: We retrospectively analyzed 24 patients with IgG4-RD with salivary gland involvement from December 2019 to December 2024. Clinical features, laboratory results, imaging findings, and treatment outcomes were evaluated.

Results: Among the 24 patients, 8 patients (33.3%) showed unilateral glandular enlargement, and 15 patients were misdiagnosed with other benign tumors and underwent surgical resection. Immunohistochemical analysis confirmed IgG4-positive plasma cells over 50 per high-power field in hotspot regions, accompanied by an IgG4+/IgG ratio greater than 40%. Elevated serum IgG4 (> 1.35 g/L) was observed in 22 patients, and some patients exhibited varying degrees of elevation in inflammatory markers. Patients with single-organ involvement have relatively lower IgG4 and pan-immune inflammation values. All 24 patients were treated with glucocorticoids, of which 6 patients with extensive systemic multiorgan involvement were treated with additional immunosuppressants. After treatment, serum IgG4 levels decreased significantly, and other inflammatory markers also showed a mild decline. Twenty-three patients improved after treatment, while 1 patient’s condition worsened owing to delayed treatment.

Conclusions: IgG4-RD involving the salivary gland had nonspecific clinical and imaging findings. Diagnosis should be comprehensively considered in combination with serum IgG4 levels and pathological examinations. Glucocorticoids are the first-line treatment drugs, and, combined with immunosuppressants, can curb recurrence and refractory disease.

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Cite

BibTeXRIS
BibTeX
@article{ding2026clinical,
  title = {{Clinical Analysis and Diagnostic Challenges of Immunoglobulin G4-Related Disease Involving the Salivary Gland: A Single Center Retrospective Analysis of 24 Cases}},
  author = {Tonghui Ding and Hongyuan Chen and Yabing Chen and Yuting Xu and Jiaming Mao and Jianjie Song and Chengjing Zhu and Wei Wang},
  journal = {Medical Science Monitor: International Medical Journal of Experimental and Clinical Research},
  year = {2026},
  month = oct,
  doi = {10.12659/msm.952641},
  url = {https://doi.org/10.12659/msm.952641},
  note = {CC BY-NC-ND 4.0},
}
RIS
TY  - JOUR
TI  - Clinical Analysis and Diagnostic Challenges of Immunoglobulin G4-Related Disease Involving the Salivary Gland: A Single Center Retrospective Analysis of 24 Cases
AU  - Tonghui Ding
AU  - Hongyuan Chen
AU  - Yabing Chen
AU  - Yuting Xu
AU  - Jiaming Mao
AU  - Jianjie Song
AU  - Chengjing Zhu
AU  - Wei Wang
PY  - 2026
DA  - 2026/10/25
JO  - Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
DO  - 10.12659/msm.952641
UR  - https://doi.org/10.12659/msm.952641
AB  - Background: Immunoglobulin G4–related disease (IgG4-RD) is a rare and complex autoimmune disorder, and there is a lack of awareness in surgical settings for patients with salivary gland involvement. This study summarized the clinical characteristics of IgG4-RD involving the salivary gland, aiming to improve clinical physicians’ understanding, diagnosis, and treatment of IgG4-RD.  Material/Methods: We retrospectively analyzed 24 patients with IgG4-RD with salivary gland involvement from December 2019 to December 2024. Clinical features, laboratory results, imaging findings, and treatment outcomes were evaluated.  Results: Among the 24 patients, 8 patients (33.3%) showed unilateral glandular enlargement, and 15 patients were misdiagnosed with other benign tumors and underwent surgical resection. Immunohistochemical analysis confirmed IgG4-positive plasma cells over 50 per high-power field in hotspot regions, accompanied by an IgG4+/IgG ratio greater than 40%. Elevated serum IgG4 (> 1.35 g/L) was observed in 22 patients, and some patients exhibited varying degrees of elevation in inflammatory markers. Patients with single-organ involvement have relatively lower IgG4 and pan-immune inflammation values. All 24 patients were treated with glucocorticoids, of which 6 patients with extensive systemic multiorgan involvement were treated with additional immunosuppressants. After treatment, serum IgG4 levels decreased significantly, and other inflammatory markers also showed a mild decline. Twenty-three patients improved after treatment, while 1 patient’s condition worsened owing to delayed treatment.  Conclusions: IgG4-RD involving the salivary gland had nonspecific clinical and imaging findings. Diagnosis should be comprehensively considered in combination with serum IgG4 levels and pathological examinations. Glucocorticoids are the first-line treatment drugs, and, combined with immunosuppressants, can curb recurrence and refractory disease.
SN  - 1234-1010
SN  - 1643-3750
ER  -