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Syed Mahmood Shah, Xiangrong Kong, Alexander Ho, Srinivas R Sadda, Beatriz E Munoz, Rupert Wolfgang Strauss, Mohamed Ibrahim, Yulia Wolfson, Sheila K West, Hendrik P Scholl, PROGSTAR study group.; Fundus Autofluorescence Characteristics in the Natural History of the Progression of Atrophy secondary to Stargardt Disease (PROGSTAR) Study. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):1652. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
To characterize fundus autofluorescence (AF) of patients with Stargardt disease (STGD) as part of the PROGSTAR study.
Baseline AF images (Heidelberg Spectralis) acquired with the reduced illuminance of autofluorescence imaging (RAFI) method of patients prospectively enrolled in PROGSTAR from 9 participating sites were graded at the Doheny Image Reading Center. Using RegionFinder™ ( Heidelberg Engineering), areas of definitely decreased AF (DDAF), well-demarcated questionably decreased AF (WD-QDAF), and poorly demarcated questionably decreased AF (PD-QDAF) were quantified. DDAF was further classified based on contiguity, location and changes at the edge of the lesion. BCVA was measured using ETDRS charts.
Eighty five eyes (49 participants) were graded to date. Demographic characteristics were as follows: 39% female, 90% white, median age of 32 (11-69) years, median age of onset was 19 (range 5-63) years, with 33% onset < 16 years; median duration of disease was 10 years (range 1-55). Median BCVA ETDRS letter score was 44 (range 23-88).<br /> Table 1 shows the AF pattern characteristics, and table 2 shows DDAF characteristics.<br /> Disease duration (DD) was positively associated with the presence of DDAF (odds ratio [OR] with 5 years increase in DD=1.5, p=0.04) and showed a trend of association with a heterogeneous background (OR=1.5, p=0.07), and was negatively associated with the presence of increased AF at lesion edge (OR=0.6, p=0.03).
FAF is an important tool for monitoring STGD disease progression. Our study suggests that patients with longer duration of disease have a higher risk of developing RPE atrophy. (i.e. areas of DDAF).
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