MULTIMODAL IMAGING OF IGG4-RELATED PAPILLITIS AND RETINAL DISEASE.

Area of Science:

• Ophthalmology
• Immunology
• Pathology

Background:

• Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition.
• Ocular involvement in IgG4-RD can manifest in various ways, but specific retinal findings are less commonly described.

Observation:

• A 52-year-old male presented with decreased vision, optic nerve edema (papillitis), and distinct grey-yellow subretinal deposits.
• Multimodal imaging, including swept-source optical coherence tomography (SS-OCT), fundus autofluorescence (FAF), and fluorescein angiography (FA), revealed characteristic findings.
• Biopsy confirmed IgG4-positive plasma cells, and elevated serum IgG4 levels supported the diagnosis.

Findings:

• The patient exhibited cuticular drusen and unique subretinal deposits on fundus examination.
• SS-OCT showed hyper-reflective subretinal material and outer retinal disruption.
• FAF demonstrated hypoautofluorescence corresponding to drusen and subretinal deposits, while FA showed hypofluorescence and optic nerve leakage.

Implications:

• This case highlights unique multimodal imaging features of IgG4-related ophthalmic disease involving the retina and optic nerve.
• The findings expand the known spectrum of ocular manifestations in IgG4-RD.
• Early diagnosis and treatment with corticosteroids can lead to resolution of ocular symptoms.