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The natural history of the progression of atrophy secondary to Stargardt disease studies (ProgStar): yearly progression rate of atrophic lesions in the retrospective study

Session Details

Session Title: Imaging I

Session Date/Time: Friday 18/09/2015 | 08:00-09:30

Paper Time: 08:48

Venue: Athena

First Author: : R.Strauss UNITED STATES

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

Abstract Details

PURPOSE:The multicenter ProgStar studies aim to characterize the natural history of Stargardt disease (STGD) and to develop new outcome measures for clinical trials. The yearly rate of progression of STGD using the growth of atrophic lesions as measured by fundus autofluorescence (AF) imaging is the primary endpoint.


Retrospective observational cohort study


FAF images from genetically confirmed STGD patients were sent from the nine participating sites to a central reading center (Doheny Image Reading Center, CA, USA) 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. Progression rates were estimated from linear mixed models with time as the independent variable. The models include random effects for the intercept, the slope of time, and the eye.


442 study eyes of 243 study participants were enrolled in the retrospective study and images from 204 eyes of 111 participants (38.4 % male, 61.6 % female) were graded for two (n=159) or three (n= 45) visits. Mean age at first visit was 31.3 years, observational time interval was 3.3 years (SD 1.5 years). At first visit, DDAF was present in 73 (36%) of the eyes, mean lesion size 3.36 (sd 4.1) mm2; WD-QDAF in 55 (27%), mean lesion size 1.35 (sd 1.69) mm2, and PD-QDAF in 122 (60%) mean lesion size 170 (sd 1.87) mm2. The estimated progression of DDAF in the 73 eyes was 0.54 mm2 per year (p<0.001). The estimated cumulative incidence of DDAF areas in the 131 eyes without DDAF at first visit was 24.4% by the end of the follow-up.


FAF may serve as a monitoring tool for interventional clinical trials in STGD that aim to slow down disease progression. Additional outcome measures for special subtypes of patients will be evaluated in the ProgStar studies.

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