Chorioretinal Findings in Intraocular IgG4-Related Disease: A Case Report and Review of the Literature.

Abstract

Purpose: This manuscript reports intraocular IgG4-related disease (IgG4-RD) presenting as panuveitis with a large subretinal mass and contralateral orbital mass. It includes a literature review highlighting IgG4-RD's diagnostic challenges, emphasizing clinical, histopathologic, and multimodal-imaging findings.

Methods: PubMed was searched for "all fields" with keywords "IgG4" AND "retina" AND "choroid" from 1974 to March 2024, revealing 16 cases of 23 eyes detailing IgG4-related chorioretinal manifestations.

Results: The most common presenting symptom was painful vision loss (31%). Chorioretinal findings included: large subretinal or choroidal masses (35%), multifocal subretinal lesions (26%), and retinal detachment (22%). Associated ophthalmic findings included: scleritis or sclerouveitis (52%), vitritis (48%), orbital disease (13%), and papillitis (9%). IgG4+/IgG ratios in ocular tissue varied 20-54.5%. IgG4+ plasma cells per high power field ranged 50-133. Ophthalmic modalities used to aid in diagnosis included B-scan ultrasonography (US-B, 56%), optical coherence tomography (OCT, 31%), fluorescein angiography (FA, 25%), fundus-autofluorescence (FAF, 13%), and indocyanine-green angiography (ICGa, 13%). Masses on B-scan were heterogenous or homogenous with medium-to-high reflectivity. Magnetic resonance imaging (MRI) evaluation was hypointense on T2-weighted imaging (75%) and hyperintense on T1-weighted imaging (38%) with progressive contrast enhancement (50%). Management required a multidisciplinary approach with steroid and steroid-sparing agents.

Conclusion: IgG4-RD has diverse and variable ocular presentations and diagnostic manifestations. Definitive diagnosis is made via biopsy. Further studies are necessary for non-invasive diagnosis.