Relative Ellipsoid Zone Reflectivity in Macular Telangiectasia Type 2

PURPOSE: The relative ellipsoid zone reflectivity (rEZR) has been proposed as an innovative biomarker for photoreceptor integrity. This study evaluates the rEZR in macular telangiectasia type 2 (MacTel) eyes of different disease stages. METHODS: The mean rEZR (ratio ellipsoid zone [EZ]/external limiting membrane [ELM] reflectivity [arbitrary units {AUs}], grey level range = 0-1) was analyzed for an entire spectral domain optical coherence tomography volume scan (global) and for each subfield of the Early Treatment Diabetic Retinopathy Study (ETDRS) grid (topographic) in patients with MacTel and controls. MacTel disease severity was classified according to Gass and Blodi. RESULTS: Linear mixed-model analysis of 145 eyes of 74 patients and 50 eyes of 25 controls revealed globally lower, yet not statistically significant, rEZR values in MacTel eyes. Topographically, most pronounced decreases were found in stages 3 and 4/5 for the temporal inner (coefficient estimates [CEs] = −25.4 [−38.2; −12.6] and −34.1 [−48.7; −19.6] AU, both: P < 0.001), the inferior inner (−29.9 [−44.6; −15.6] and −35.3 [−52.1; −18.5] AU, both: P < 0.001), the nasal inner (−21.5 [−35.52; −7.4] and −31.6 [−47.6; −15.6] AU, P = 0,003 and P < 0.001), and in the superior inner subfield of stage 4/5 (−25.0 [−42.0; −7.9] AU, P = 0.004). CONCLUSIONS: The rEZR showed association with disease severity and the predilection area of MacTel. Given the current understanding of the pathophysiological concept of MacTel, these findings underscore the value of the rEZR as a potential novel biomarker for outer retinal integrity. Longitudinal studies are demanded to better characterize its value as a biomarker for early photoreceptor alterations and disease progression in MacTel.