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Christopher Hampton, Khayyam Durrani, Jeanine Suchecki, Stanley David Hudnall, David Waitzman; IgG4-Related Disease Presenting as Bilateral Lacrimal Gland Enlargement in a Patient with a 24-Year History of Undiagnosed Systemic Illness. Invest. Ophthalmol. Vis. Sci. 2017;58(8):1795.
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Immunoglobulin G4-related disease (IgG4-RD) is an under-recognized cause for lacrimal gland enlargement. A 43-year old Caucasian woman presented with vague, poorly described visual symptoms and bilateral lacrimal gland enlargement. Excisional biopsy demonstrated positive IgG4-RD.
Case report based on review of medical records.
A 43-year old woman with a 24-year history of hyperplastic lymphoid lesions excised from the right upper lobe of her lung, left submandibular gland, left axillary lymph node, and left lacrimal gland, presented with increasing trouble with vision over two years. She complained of disorientation, nausea, inability to focus, and dizziness while driving secondary to a sense of self-motion. She denied headaches and diplopia. Her visual exam showed mild ptosis of the right upper eyelid, but was otherwise unremarkable. Excisional biopsy of the left lacrimal gland two years prior to presentation was interpreted as “florid reactive lymphoid hyperplasia.” Evaluation for malignancy, infection, and inflammatory disease showed negative BCL-2 reactivity, no EB virus titers, no B-cell clonality, and negative genetic testing for B-cell neoplasms. Repeat imaging of the orbits following our exam showed progressive enlargement of the opposite lacrimal gland suggesting that IgG4-RD was a possibility (well described in Japanese literature). The previous left lacrimal gland specimen was then retrieved. Staining with IgG4 specific antibody demonstrated greater than 50% of the plasma cells to be IgG4+. A complete resolution of visual disturbances and reduced lacrimal gland enlargement was attained with Rituximab.
Two aspects of this case are critical in evaluating patients with ocular symptoms associated with systemic illness. The first is that IgG4-RD should be regularly included in the differential diagnosis of lacrimal gland enlargement, especially if bilateral. Second, patients with poorly characterized visual symptoms of motion sensitivity, generalized malaise, and/or a history of lesions of “lymphoid hyperplasia” may in fact have an undiagnosed systemic illness. Definitive diagnosis of the ocular pathology not only can result in the choice of immuno-therapy that can lead to dramatic reduction of the ocular disease, but also many of the “non-specific” systemic symptoms.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.
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