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The International Journal on Orbital Disorders, Oculoplastic and Lacrimal Surgery
Volume 43, 2024 - Issue 3
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Case Report

Probable IGG4 related ophthalmic disease presenting with uveitis

, , , , , & show all
Pages 354-358 | Received 19 Aug 2022, Accepted 02 Oct 2022, Published online: 17 Oct 2022
 

ABSTRACT

We present a case of an uncommon presentation of IgG4-related ophthalmic disease (ROD). A 58-year-old female presented with unilateral acute anterior uveitis of the right eye, which progressed to scleritis with the development of an associated orbital mass despite treatment with oral glucocorticoid. Initial histopathology of an orbital biopsy was non-diagnostic and continued progression of the disease lead to complete loss of vision in the right eye. The development of uveitis in the previously unaffected left eye led to the decision for enucleation of the right globe and further orbital biopsy. Histopathology revealed features supporting IgG4-related ophthalmic disease. Oral glucocorticoid therapy failed to induce remission, and rituximab therapy was initiated, leading to a rapid resolution in her symptoms. Other cases with a similar presentation report a poor visual prognosis, highlighting the need for prompt diagnosis and treatment of uveitis associated with signs of orbital or scleral involvement.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the article.

Patient consent

The patient described has granted consent for the use of their data and clinical images.

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Funding

The authors report that there is no funding associated with the work featured in this article.

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