Choroidal Effusion as an Ocular Manifestation of Immunoglobulin G4–related Disease

Abstract

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.

Multimodal Imaging of Igg4-Related Papillitis and Retinal Disease

“…In other reports describing patients presenting with IgG4related retinal and choroidal lesions subsequently underwent lumbar puncture and flow cytometry analysis. 1,3,4 Similarly, our case did not have evidence of vitritis, and lumbar puncture and flow cytometry were unremarkable, decreasing the likelihood of intraocular lymphoma. In cases of sarcoidosis, granulomatous inflammation would be expected and also observed on histopathology of other organs.…”

“…Interestingly, the sex ratio for IgG4-related autoimmune pancreatitis is 3:1 men to women, whereas head-and neck-related disease has roughly equal proportions between men and women. 1,3,4 A Japanese study evaluating IgG4-ROD in 65 patients found that the lacrimal gland was the most involved orbital structure (88%). Other IgG4related lesions were also found to involve the trigeminal nerve (39%), extraocular muscles (25%), Supported by the Macula Foundation Inc, New York, NY.…”

“…1,5,6,11 Patients may remain on long-term steroids with relapse being common upon cessation. [1][2][3][4]7,[12][13][14][15] In patients who have disease refractory to corticosteroids, recent studies also demonstrated that B-cell depletion by using rituximab, an anti-CD20 antibody, has also been efficacious. This is likely because depletion of B-cells prevents further differentiation into plasma cells that produce IgG4.…”

“…1,2 Rarely, IgG4-ROD has been described regarding intraocular involvement with the retina and choroid. 1,3,4 We describe multimodal imaging and evaluation in a patient with IgG4-related papillitis and retinal involvement, which expand the spectrum of known IgG4-ROD findings.…”

Immunoglobulin G4-related ophthalmic disease presenting as a steroid-resistant choroidal mass: a case report

“…There are six reported cases of IgG4-related choroidal mass 2-7 and one reported case of choroidal effusion. 8 Our case is the first case that demonstrates sequential but heterogeneous involvement of the fellow eye, which developed anterior uveitis while on high-dose steroids. With the prompt commencement of rituximab and second-line immunosuppressants, the condition of the fellow eye was brought under control with visual acuity preserved.…”