Choroidal Effusion As An Ocular Manifestation of Immunoglobulin G4-Related Disease

Purpose:To report a unique ocular manifestation of immunoglobulin G4-related disease (IgG4-RD) as choroidal effusion.Methods:Case report.Results:An 81-year-old man presented with clinical manifestations of choroidal effusion, confirmed by fluorescein angiography, ophthalmic ultrasound (posterior segment), high-frequency ultrasound biomicroscopy, Heidelberg Spectralis spectral domain optical coherence tomography, and enhanced depth imaging spectral domain optical coherence tomography. Systemic multi-organ findings included chronic pancreatitis, irregularity of the intrahepatic bile ducts, lymphadenopathy, aortitis, fibrotic changes of the lungs, hypophysitis, and sialadenitis. Extensive workup revealed elevated IgG4 serum levels, and lymph nodes biopsy showed reactive pattern with polyclonal IgG4 positive plasma cells. These findings were compatible with IgG4-related disease. Treatment with oral corticosteroids resulted in complete resolution of the choroidal effusion.Conclusion:Choroidal effusion may be an ocular manifestation of IgG4-related disease. Oral corticosteroids is an effective treatment. Ophthalmologists should be aware of this possible finding.