Progression of atrophic lesions determined by fundus autofluorescence: The natural history of the progression of atrophy secondary to stargardt disease (ProgStar) study

Session Details

Session Title: Free Paper Session 4: AMD II

Session Date/Time: Thursday 07/09/2017 | 11:00-12:30

Paper Time: 12:12

Venue: Room 111

First Author: : R.Strauss AUSTRIA

Co Author(s): :    X. Kong   M. Michaelides   A. Ho   S. Sadda   S. West   H. Scholl              

Abstract Details

Purpose:

The multicentre ProgStar study aims to characterize the natural history of Stargardt disease type 1 (STGD1) and develop new outcome measures for trials. The yearly rate of growth of atrophic lesions measured by fundus autofluorescence (FAF) imaging is the primary endpoint.

Setting:

Prospective, multicentre, natural history cohort study conducted in the USA, United Kingdom and Europe

Methods:

Participants were enrolled from nine sites in the US and Europe, and were followed every 6 months. 18-month follow-up data were used in this analysis. FAF images were sent from the nine participating sites to a central reading centre and areas of definitely decreased AF (DDAF), well-demarcated questionably decreased AF (WD-QDAF), and poorly demarcated questionably decreased AF (PD-QDAF) were outlined and quantified. Based on background uniformity, homogeneous versus heterogeneous background was defined. Linear mixed effects models were used to estimate the annual rate of change in lesion areas while accounting for between-eye and within-eye correlations.

Results:

489 study eyes of 259 patients (54 % female) were enrolled in the prospective study. Mean age at baseline was 33.3 (sd 15.1) years. At baseline, DDAF was present in 307 (63%) eyes, mean lesion size 3.94 (sd 4.36) mm2; WD-QDAF in 71 (15%) eyes, mean lesion size 1.54 (sd 1.38) mm2, and PD-QDAF in 300 (62%) eyes, mean lesion size 2.16 (sd 1.91) mm2. Over 18 months, 50 eyes out of the 151 eyes (33.1 %) without any DDAF at baseline and available at month 18, developed a lesion of DDAF. Among eyes with DDAF at baseline, progression rate of DDAF was 0.75 (95%CI 0.61-0.90) mm2 per year (p<.001), and the rate of DDAF growth was significantly different by baseline AF background (p<.001): it was 0.44 (0.23-0.65) and 1.02 (0.83-1.21) mm2 per year in eyes with homogeneous versus heterogeneous background, respectively. Baseline DDAF size was also significantly associated with DDAF growth rate - the larger the baseline lesion, the faster the progression (p<.001). Combining all lesion types, the mean progression rate was 0.65 (CI 0.55-0.74) mm2 per year.

Conclusions:

FAF may serve as an outcome measure of treatment trials that aim to slow/halt the progression of STGD1.

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