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Histopathologically Proven IgG4-Related Orbital Disease: A Two-Tertiary Center Study from Turkiye
Burak Ulas1, Altan Atakan Özcan1, Melis Palamar2
1Faculty of Medicine Ophthalmology Department, Cukurova University, Adana, Turkiye.
Ocular Immunology and Inflammation
|June 18, 2025
Summary
This study highlights IgG4-related orbital disease (IgG4-ROD) clinical features. Lacrimal gland involvement and eyelid swelling are common, with most patients responding well to glucocorticoid treatment.
Area of Science:
- Ophthalmology
- Immunology
- Pathology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition affecting various organs.
- IgG4-related orbital disease (IgG4-ROD) is a manifestation of IgG4-RD affecting the orbit.
- Accurate diagnosis and understanding of clinical presentations are crucial for effective management.
Purpose of the Study:
- To evaluate the clinical features and symptoms of IgG4-related orbital disease (IgG4-ROD).
- To analyze demographic profiles, clinical findings, and ocular symptoms in a cohort of IgG4-ROD patients.
Main Methods:
- Retrospective review of medical records from 20 patients with histopathologically proven IgG4-ROD.
- Analysis of demographic data, clinical presentations, and ocular symptoms.
- Inclusion of patients from two tertiary referral university hospitals in Turkey.
Main Results:
- The mean age of patients was 40.9 years, with 65% females.
- Lacrimal gland involvement (84.2%) was the most frequent manifestation, followed by orbital soft tissues (52.6%).
- Eyelid swelling and redness were the most common symptoms; all patients responded to glucocorticoids, with one relapse.
Conclusions:
- IgG4-ROD presents with diverse clinical features that can overlap with other orbital conditions.
- Suspecting IgG4-ROD in cases of orbital involvement, particularly with eyelid swelling and redness, is important.
- Biopsy is essential for accurate diagnosis of IgG4-ROD.
