Abstract Listings 2024

Purpose

This study aims to delineate the clinical radiologic and histopathologic characteristics of IGG4 related orbital disease in Saudi Arabia, in two tertiary institutions.

Methods

This is a retrospective multi-centric study between the years 1983 – 2023. All patients underwent lacrimal gland biopsy and imaging, as well as histopathologic analysis. The inclusion criteria are high serum igg4, and abundant infiltration of igg4 positive plasma cells/high power field.

Results

The study cohort comprised 10 females and 5 males. The mean disease onset was 5.50 years ± 3.62 (range: 0.31 – 10.1 years). Eleven patients (73%) had full extraocular movement, while the remaining four patients had varying degrees of limitation. Orbital manifestations included eyelid swelling (47%), lacrimal gland swelling (47%), conjunctival swelling, tenderness, soft tissue swelling, optic nerve edema, and painful presentation (47%). Extra orbital involvement with swelling in the parotid gland, another patient experienced proximal muscle weakness, one patient suffered from vitiligo, while the final patient had adult xanthogranuloma. Increased numbers of IgG4-positive plasma cells were observed in 53%, and 40% had a high IgG4/IgG ratio. Histopathological findings revealed the prevalence of plasma cells in all study participants, while lymphoid follicles were present in 60% and fibrosis in 40%. Systemic treatment in the form of steroids and rituximab was administered to 60% and the remaining 6 patients resolved spontaneously.

Conclusion

Our data demonstrates a clear female predominance. Eyelid swelling and lacrimal gland swelling, were the most common signs. Radiologically, we note the presence of olfactory and infraorbital nerve enhancement which is a well documented finding. Sclerosing igG4 disease in association with lymphoid hyperplasia and subsequent lymphoma is an expected sequelae. Many therapies have been described all with positive outcomes.
Prompt diagnosis is essential in initiating systemic treatment, preventing blindness, disease progression, and relapse.

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