CHORIORETINAL FINDINGS IN INTRAOCULAR IGG4-RELATED DISEASE: A CASE REPORT AND REVIEW OF THE LITERATURE
Maja Magazin1, Skyler G Jones1, Alexis Fazio2
1Department of Ophthalmology, Medical College of Georgia, Augusta University, Augusta, Georgia; and.
Retinal Cases & Brief Reports
|February 20, 2025
Summary
Immunoglobulin G4-related disease (IgG4-RD) can present with varied ocular symptoms, including vision loss and retinal masses. Diagnosis often requires biopsy, highlighting challenges in non-invasive identification of this condition.
Area of Science:
- Ophthalmology
- Immunology
- Pathology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition with diverse manifestations.
- Intraocular involvement of IgG4-RD can mimic other orbital and uveitic conditions, posing diagnostic challenges.
Purpose of the Study:
- To report a case of intraocular IgG4-related disease (IgG4-RD) presenting as panuveitis with a large subretinal mass and contralateral orbital mass.
- To review the literature on IgG4-RD's diagnostic challenges, emphasizing clinical, histopathologic, and multimodal-imaging findings.
Main Methods:
- A comprehensive literature search was conducted using PubMed with keywords "IgG4", "retina", and "choroid" from 1974 to March 2024.
- Analysis included 16 cases involving 23 eyes with IgG4-related chorioretinal manifestations.
Main Results:
- Painful vision loss was the most common symptom (31%). Ocular findings included subretinal/choroidal masses (35%), retinal detachment (22%), scleritis/sclerouveitis (52%), and vitritis (48%).
- Multimodal imaging (B-scan US, OCT, FA, FAF, ICGA) and MRI revealed characteristic findings for masses and orbital involvement.
- Histopathologic analysis showed variable IgG4+/IgG ratios and elevated IgG4+ plasma cells.
Conclusions:
- IgG4-RD exhibits diverse ocular presentations and diagnostic features.
- Definitive diagnosis of ocular IgG4-RD relies on histopathologic confirmation via biopsy.
- Further research is needed to develop non-invasive diagnostic methods for IgG4-RD.
