Rituximab for Relapsing Igg4-Related Ophthalmic Disease Resistant to Corticosteroids: Literature Review and a Case Report Publisher

Summary: Can an eye tumor hide behind normal IgG4 blood levels? A case report found tissue-confirmed orbital disease despite normal serum IgG4, with improvement on rituximab. #EyeHealth #RareDisease

Habibi Moini S ; Kabiri S ; Behbood A ; Sefidian R ; Mohammadianinejad S E ; Harirchian M H
Source: Current Journal of Neurology Published:2025

Abstract

Background: Immunoglobulin G4 (IgG4)-related ophthalmic disease (IgG4-ROD) is a newly recognized term for patients with IgG4-related disease (IgG4-RD) with ocular adnexal involvement. It primarily affects the orbit, lacrimal glands, extraocular muscles, and other periocular structures, leading to orbital masses, proptosis, and potential vision impairment. This study aims to present a case of bilateral IgG4-ROD to highlight its diagnostic approaches and therapeutic options. Case Report: We report a case of 34-year-old woman presenting with bilateral proptosis. Imaging revealed involvement of lacrimal glands as well as a right retrobulbar mass encasing ocular muscles. Despite having a normal serum IgG4 level (28 mg/dl), the diagnosis of IgG4-ROD was made based on presence of ophthalmic tissue mass and IgG4-positive plasma cells in histopathology (> 40%). Recurrence of periorbital symptoms occurred five months after initial remission while being on glucocorticoids (GCs) and azathioprine (AZA). Conclusion: Symptoms improved after being switched to rituximab (RTX), and the patient remained symptom-free during a one-year follow-up. IgG4-ROD can manifest as a pseudotumor with involvement of adjacent tissues and should be considered in approaching ocular lesions. Appropriate workup to detect multi-organ involvement is essential. Copyright © 2025 Iranian Neurological Association, and Tehran University of Medical Sciences.