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Xiangrong Kong, Rupert Wolfgang Strauss, Beatriz E Munoz, Yulia Wolfson, Ann M Ervin, Srinivas R Sadda, Sheila K West, Hendrik P Scholl, The ProgStar study group; The Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) Study: Associations between Fundus Autofluorescence and Microperimetry at Baseline of the Prospective Study. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):2584. doi: https://doi.org/.
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The multi-center ProgStar studies aim to characterize the natural history of Stargardt disease (STGD) and establish structure-function correlations. The associations between characteristics of fundus autofluorescence (AF) and those of microperimetry (MP) at the prospective study baseline were assessed.
Genetically confirmed STGD patients were enrolled from nine participating sites. Their AF and MP images were graded by the Doheny Image Reading Center. Areas of definitely decreased AF (DDAF), well-demarcated questionably decreased AF (WD-QDAF) and poorly-demarcated AF (PD-QDAF) were quantified. The mean sensitivity (MS) and the numbers of points with absolute scotoma (AS) and relative scotoma (RS) were assessed in photopic microperimetry using a 10-2 test algorithm. Linear models with generalized estimating equations were used to assess the associations between AF and MP measures, adjusting for potential between-eye correlation.
To date AF images and MP-1 exams were graded for 64 study eyes of 39 patients. Mean age at time of enrollment was 34 (range 11-68) years, and the median age at disease onset was 23 (range 5-63) years. N=29 eyes (45%) had DDAF, with a mean lesion size (MLS) of 5.4 (standard deviation [SD] 5.9) mm2; N=23 eyes (36%) had WD-QDAF with MLS of 2.0 (SD 2.4) mm2; and N=42 eyes (45%) had PD-QDAF, with MLS of 2.0 (SD 1.8) mm2. Mean MS was 11.8 (SD 5.4) dB. The mean numbers of points of AS and RS were 14.9 (SD 18.3) and 8.6 (9.7), respectively.<br /> An increase of 1 mm2 in MLS of DDAF was associated with a decrease of MS of 0.61 dB (p< .0001) and also with an increase in 2.2 points of AS (p<.0001), but not with RS (p=0.62). WD-QDAF was not significantly associated with MS (p=0.99) or AS (p=0.62), but a 1 mm2 increase in WD-QDAF was significantly associated with a decrease of 1.1 points in RS (p<.0001). PD-QDAF did not show significant correlation with any MP measures (p=0.28 for MS, p=0.19 for AS and p=0.08 for RS).
Area of DDAF, which is the main outcome of ProgStar, was associated with a functional loss as detected by MP. Different subtypes of structural changes in AF may have different functional consequences in MP. The reported correlations may be important for the design of interventional clinical trials in STGD.
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