Clinical Analysis and Diagnostic Challenges of Immunoglobulin G4-Related Disease Involving the Salivary Gland: A Single Center Retrospective Analysis of 24 Cases
Year: 2026
Type: article
Study design: Observational Study
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 AND 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.
Source: Medical Science Monitor
DOI: 10.12659/msm.952641
Institutions: Second Military Medical University, Changhai Hospital
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
OpenAlex ID: W7204821535