Recovery of vision following treatment with systemic corticosteroid in a case of IgG4-related eye disease after prolonged neglect Yuki Abe 1 , Norihiko Kitaya 1 , Yuji Kanisawa 2 1Department of Ophthalmology, Oji General Hospital 2Department of Hematology, Oji General Hospital pp.822-830
Published Date 2020/7/15
DOI https://doi.org/10.11477/mf.1410213610
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Abstract Purpose:To report a case who had been misdiagnosed as mucosa-associated lymphoid tissue lymphoma for 28 years and who improved after systemic glucocorticoid for IgG4-related eye disease.

Case:A 72-year-old male presented with exophthalmos and impaired visual acuity in both eyes. He had been diagnosed with meibomitis and been treated with systemic prednisolone 28 years before. He was diagnosed as pseudolymphoma 3 years later and as bilateral orbital tumor another 5 years later. Histopathological studies led to the diagnosis of MALT lymphoma and to treatment by radiation. He showed bilateral proptiosis and enlarged orbital tumor another 5 years later. Six courses with R-CHOP therapy was followed by improvement. He was then referred to us for eye manifestations.

Findings and Clinical Course:Corrected visual acuity was 1.0 in either eye. He showed no proptosis. Orbital tumor in both eyes recurred 7 and 10 years later. Computed tomography showed enlargement of orbital tumor. Hematological studies showed elevated level of IgG4. Biopsy of submandibular gland showed IgC4-related ocular disease. Peroral prednisolone followed with improved visual acuity.

Conclusion:IgG4-related disease is a new concept reported in 2011. In ophthalmology, it may manifest as lacrimal adenitis and intraorbital mass. The present case illustrates that it may simulate MALT lymphoma and may respond to systemic corticosteroid even after long neglect.

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