Histopathologically Proven IgG4-Related Orbital Disease: A Two-Tertiary Center Study from Turkiye.

Aim: The purpose of this study was to evaluate the clinical features and symptoms of IgG4-related orbital disease (IgG4-ROD) cases from two ophthalmology clinics.

Methods: The medical records of 20 patients with histopathologically proven IgG4-ROD diagnosed at 2 tertiary referral university hospitals in Turkey were retrospectively reviewed. The demographic profiles, clinical findings, and ocular symptoms of the patients were analyzed.

Results: The mean patient age was 40.9 ± 20.1 (5-67) years (65% female, 35% male). IgG4-ROD involvement was bilateral in 25% of cases. The involvement sites were lacrimal gland (84.2%), orbital soft tissues (52.6%), conjunctiva (15.8%). Multiple ophthalmic sites of involvement were detected in seven patients. Lacrimal gland involvement was the most frequent ophthalmic manifestation of IgG4-ROD. The most common complaints of the patients were swelling and redness of the eyelids. All patients responded to glucocorticoid treatment. However, one patient (5.3%) relapsed and required long-term corticosteroid and immunosupressive agent use.

Conclusion: IgG4-ROD can present with overlapping features of clinical history, physical examination, and imaging findings. Therefore, in any orbital involvement - especially with eyelid swelling and redness, it is important to suspect IgG4-ROD and to perform biopsy to achieve the accurate diagnose.