Multimodal Imaging of Igg4-Related Papillitis and Retinal Disease

Abstract

Purpose: To report a case of IgG4-related ophthalmic disease, which presented with papillitis and subretinal deposits.Methods: Observational case report with multimodal imaging.Results: A 52-year-old man with a history of persistent lymphadenopathy presented with decreased vision in his left eye. Funduscopic examination demonstrated cuticular drusen in both eyes and florid edema of the left optic nerve, along with scattered circumscribed greyyellow subretinal deposits that were distinct from the cuticular drusen. Swept-source optical coherence tomography demonstrated a hyper-reflective subretinal material corresponding to the grey-yellow subretinal deposits on clinical examination along with diffuse outer retinal disruption. Fundus autofluorescence revealed scattered hypoautofluorescence corresponding to cuticular drusen and also larger patches of hypoautofluorescence corresponding to the grey-yellow subretinal deposits. Fluorescein angiography demonstrated hypofluorescence corresponding to the large subretinal deposits and leakage at the optic nerve. Lymph node biopsy demonstrated IgG4-positive plasma cells and elevated serum IgG4 levels leading to a diagnosis of IgG4-related ophthalmic disease. The patient was treated with oral prednisone with subsequent resolution of the optic nerve edema. Conclusion:We describe multimodal imaging of unique retinal and optic nerve findings associated with IgG4-related ophthalmic disease. Our report broadens the spectrum of ocular involvement associated with IgG4-related disease.

IgG4-related hypertensive granulomatous anterior uveitis

“…Prior cases have described findings of optic nerve head edema, exudative retinal detachments, hypopyon, and multifocal as well as solitary choroidal lesions. 5 , 6 All cases have also been associated with scleral and/or orbital inflammation, with the exception of one patient who presented with an isolated panuveitis. 7 Additional investigations to consider in future cases would be indocyanine green angiography, which could reveal potential choroidal involvement that would otherwise not observed on clinical examination.…”

Immunoglobulin G4–related Ophthalmic Disease Mimicking Intraocular Lymphoma: A Case Report

“…10 Descriptions of intraocular findings are more rare and are counted as papillitis, panuveitis, and choroidal effusion. 11,12 Gange et al 9 described a case of choroiditis in a 64-year-old man, with a medical history positive for IgG4-RD, presenting multiple, bilateral choroidal lesions.…”

Diagnostic and Therapeutic Challenges

“…Although prior case series have reported orbital inflammatory complications, and ciliary body tumor associated with IgG4-RD, reports of retinal and posterior segment complications are rare. [15][16][17][18] Reports of orbital disease have primarily described lacrimal gland, soft tissue, extraocular muscle, and optic nerve disease, which have been treated with glucocorticoids, conventional disease-modifying antirheumatic drugs, and more recently rituximab. 19 These strategies are considerations for the patient described by Wang et al, potentially with ongoing monitoring of plasma levels of IgG4, plasmablasts, and clinical follow-up for evidence of systemic involvement with a multidisciplinary approach.…”