Abstract ID: 26-582
Clinicopathological Characteristics in Orbital IgG4-Related Disease: A Retrospective Case Series
Author: Antonia Vieira Base Hospital / Institution: Pontificia Universidad Católica de Chile
Presentation Type: ePoster Presentation
Purpose
To describe the clinical and histopathological characteristics of patients with orbital IgG4-Related Disease (IgG4-RD) and explore potential clinicopathological associations, including USP25 expression patterns.
Methods
A retrospective review was performed of patients with histopathological diagnosis of orbital IgG4-RD at a tertiary referral centre. Demographic, clinical, radiological, serological and histopathological variables were analysed.
Results
Twenty-nine patients were identified. Mean age at diagnosis was 54 years, with a slight female predominance (51.7%). The lacrimal gland was the most frequently affected site. Bilateral disease was present in 17.2% of patients, while extraocular involvement was identified in 10.3%. Elevated serum IgG4 levels did not clearly correlate with disease extent. Patients with bilateral disease showed a higher frequency of systemic involvement compared with unilateral cases (20% vs 8.3%). Histopathological analysis demonstrated dense lymphoplasmacytic infiltration with variable fibrosis. Germinal centre formation was observed in 69.2% of evaluable biopsies, eosinophilic infiltrates in 63.6%, storiform fibrosis in 30.8%, and pronounced architectural distortion in 76.9%. Obliterative phlebitis was absent in all specimens.USP25 expression demonstrated heterogeneous staining patterns across inflammatory, stromal and epithelial compartments. Higher USP25 expression appeared more prominent in biopsies with denser inflammatory infiltrates and higher fibrosis grades, suggesting a relationship with chronic inflammatory activity and tissue remodelling.
Conclusion
Orbital IgG4-RD demonstrates heterogeneous clinicopathological manifestations, most commonly affecting the lacrimal gland and orbital soft tissues. Germinal centre formation, eosinophilia and pronounced architectural distortion were frequent findings, whereas obliterative phlebitis was uncommon. Variable USP25 expression may reflect inflammatory and fibrotic activity, supporting further investigation as a potential biomarker in IgG4-RD.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Josefina | Fry | Pontificia Universidad Católica de Chile |
| Afaf | Rumie | Pontificia Universidad Católica de Chile |
| Roberto | Arellano | Pontificia Universidad Católica de Chile |
| Pablo | Zoroquiain | Pontificia Universidad Católica de Chile |