IgG4-related disease and lymphoma in the ocular adnexa

Micheal O'Rourke, Thomas Hardy, Penny McKelvie, Alan McNab

Meeting:  2022 RANZCO


Date:      -

Session Title: FREE PAPERS – Oculoplastic/Ocular Oncology

Session Time:      -

Purpose: IgG4-related disease (RD) is a chronic fibro- inflammatory disease forming masses in multiple sites including the orbit. Chronic inflammatory diseases increase the risk of development of lymphoma. We describe our series of ocular adnexal lymphoma occur- ring in the context of IgG4-RD.

Methods: A retrospective review of the histopathol- ogy database was used to identify patients matching the criteria of IgG4-RD and lymphoid hyperplasia or lymphoma from 2014 to the present time. Patient demographics, disease location and outcomes were recorded.

Results: Extra-nodal marginal zone mucosal-associated lymphoid tissue -type lymphoma was found in 12 patients. There was an equal sex distribution with mean age 52.3 years (SD 13.7). Diagnosis was based on five lacrimal gland, six orbital and one infraorbital nerve biopsies. A preceding histological diagnosis of IgG4-RD occurred in eight patients of which three had a history of reactive lymphoid hyperplasia (mean duration 18 months prior to development of lymphoma). Preceding submandibu- lar and lacrimal gland disease diagnosed as Sjogren’s disease occurred in one patient. IgG-4 secreting lym- phoma occurred in four patients. Serum IgG4 was raised in five patients. Extra-ocular organ involvement occurred in one patient. All patients underwent treat- ment as directed by their haematologist with good outcomes.

Conclusion: IgG4 RD lymphoma is usually extra- nodal marginal zone mucosal-associated lymphoid tissue type and can occur in the setting of pre- existing fibro-inflammatory disease or de novo IgG-4 secreting lymphoma which can appear clinically indistinguishable. Serum IgG4 does not differentiate neoplastic from IgG4-RD. Outcome from IgG-4 related lymphoma of the ocular adnexa is favourable.