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Kathryn L. Pepple, Michael Cusick, Glenn J. Jaffe, Prithvi Mruthyunjaya; SD-OCT and Autofluorescence Characteristics of Autoimmune Retinopathy. Invest. Ophthalmol. Vis. Sci. 2011;52(14):4284.
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To identify the SD-OCT and fundus autofluorescence (AF) characteristics found in patients diagnosed with autoimmune retinopathy (AIR). Identification of abnormal retinal structure with high-resolution imaging could help improve our understanding of disease pathogenesis.
Retrospective review of patients diagnosed with AIR at the Duke Eye center from January 1, 2005 thru September 30, 2010. Patients were included that demonstrated bilateral visual field loss, had abnormal electroretinograms (ERGs), had serum autoantibodies against retinal antigens demonstrated on western blot, and had SD-OCT and AF imaging of the fundus obtained at the time of presentation. Imaging studies were reviewed and the group of patients with abnormal findings was compared to the normal imaging cohort. Patient demographic data including medical history and autoantibody profile were tested for correlation with abnormal imaging.
Seven patients were identified that met the inclusion criteria. The average age was 60 years (range 48 to 73) and four (57%) were female. Five (71 %) had a prior or recent diagnosis of cancer. Four (57%) patients were identified with both AF and SD-OCT abnormalities. In these patients, there was central macular hypo-autofluorescence surrounded by a ring of hyper-autofluorescence and SD-OCT evidence of loss of the photoreceptor layer with sparing of the central macula. The boundary of abnormal AF corresponds to the boundary of photoreceptor layer loss on SD-OCT. Three autoantibodies (30kDa, 40kDa, and 46kDa) showed a correlation to the presence of imaging abnormalities. No correlation was found with the presence or type of underlying malignancy.
This is the first study to identify both SD-OCT and AF imaging abnormalities in patients with AIR. We also identified retinal ultra-structural changes that support photoreceptor cell death as a mechanism of disease. In addition, FAF changes suggest that retinal autoantibodies may also impact RPE health and function concurrently or indirectly through photoreceptor loss.
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