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Avni P Finn, Akshay Thomas, Dilraj Singh Grewal, Glenn Jay Jaffe; Cystoid Macular Edema as a Marker in the Progression of Autoimmune Retinopathy. Invest. Ophthalmol. Vis. Sci. 2017;58(8):554.
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Autoimmune retinopathy (AIR) is a rare disorder of the retina characterized by vision loss, visual field deficits, and photoreceptor dysfunction in the presence of anti-retinal antibodies. Cystoid macular edema (CME) may be seen in patients with this disease. This study aimed to elucidate the relationship between CME and disease progression relationship by using ellipsoid zone (EZ) length as a surrogate for disease severity, and correlating this to the presence of CME.
A retrospective chart review of AIR patients presenting between 2008 and 2016 was conducted. A single reader determined the presence or absence of CME at each visit using the volume scan and measured the preserved ellipsoid zone (EZ) length on the centermost 9 mm SD-OCT 7-line volume B scan. A second reader confirmed the EZ borders on equivocal images. All scans were of good quality and shadowing did not affect the ability to calculate EZ length.
14 patients (28 eyes) were included in this study, 8 males and 6 females with average age at presentation of 59 years and mean follow-up of 38 months. All patients had positive anti-retinal antibodies, evidence of ERG dysfunction, and a negative malignancy evaluation. In 8 eyes that did not have CME at initial presentation and did not develop CME, the mean EZ length was 6083 microns at initial visit and 6014 microns at final follow-up. In 17 eyes that had CME at initial presentation, the mean EZ length was 3020 microns at initial presentation and 2233 microns at final follow-up (p=0.05). The difference in initial EZ length and final EZ length in eyes that did not have CME and did have CME was significant, p=0.002 and p=0.0001, respectively. In 3 eyes that did not have CME at initial presentation but developed CME during the course of their disease, the mean EZ length was 5570 microns at initial presentation and 2351 microns at final follow-up. Additionally, in these 5 eyes, an average decrease in EZ length of 1400 microns was seen between the visit prior to the development of CME and after the development of CME.
Eyes without CME have longer EZs than those that do. Furthermore, in eyes that previously did not have CME there was a disease progression inflection point as manifested by a marked decrease in EZ length after CME developed. While this study is limited by its small sample size, the development of CME may suggest more advanced disease in patients with AIR.
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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