A 58-year-old male presented with bilateral periorbital swelling, ptosis, and lacrimal gland enlargement (Figure 1A). IgG4-related disease is a systemic immune-mediated condition that can affect multiple organs, including orbital structures; however, lacrimal gland involvement is relatively uncommon, particularly in Eastern countries^(1,2). Diagnosis is based on imaging and histopathological examination, which typically demonstrate dense lymphocytic infiltration, fibrosis, and IgG4-positive plasma cells (Figure 1B–D), allowing exclusion of conditions such as Mikuliz disease⁽³⁾, Sjögren's syndrome, lymphoma, and idiopathic orbital inflammation⁽³⁾. Management generally includes systemic corticosteroids, immunosuppressive therapy, and careful long-term follow-up^(1,3).

REFERENCES

1. Huang S, Juniat V, Satchi K, Cohen LM, Davis G, Rootman DB, et al. Bilateral lacrimal gland disease: clinical features and outcomes. Eye (Lond). 2022;36(11):2163-71.

2. Ito M, Vaidya A, Kakizaki H, Takahashi Y. Spontaneous regression of immunoglobulin G4-related dacryoadenitis and multiple organ involvement: A case report. Medicine (Baltimore). 2023;102(1):e32618.

3. Bae DH, Kim YR, Yang W, Kim GI, Lew H, Yoo J. Function of immune checkpoints in IgG4–related disease with lacrimal gland involvement: clinical features, serum igG4 level, immunohistochemical landscape, and treatment responses. Int J Mol Sci. 2025;26(7):3021.

Data Availability Statement: The datasets generated and/or analyzed during the current study are already available.

Edited by:
Editor-in-Chief: Newton Kara-Júnior

Submitted for publication: December 18, 2025.
Accepted for publication: January 21, 2026.

Funding: This study received no specific financial support.

Disclosure of potential conflicts of interest: The authors declare no potential conflicts of interest.