About the Study

Study Worker

Sponsored by the Foundation Fighting Blindness, the Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) studies are comprised of retrospective and prospective longitudinal observational studies. The retrospective study includes clinical examination findings and images collected between 2008 (and earlier) and 2014 and progression will be retrospectively evaluated. The prospective study consists of a 24 month observational period from 2013/2014 for two years, with one visit every six months.

The study population will include 250 STGD patients in the retrospective study and 250 patients in the prospective study recruited currently at 9 clinical centers across the U.S. and Europe. The enrollment of a large population of children and adults with STGD will assist in evaluating efficacy measures for future clinical trials. The outcomes of interest are measured via imaging (i.e., spectral-domain optical coherence tomography, fundus autofluorescence) and psychophysical testing (i.e., visual acuity, microperimetry).

Images are evaluated by a central Reading Center at the Doheny Eye Institute, Los Angeles, California. Data Coordinating Center is located within the Dana Center for Preventive Ophthalmology, Johns Hopkins University School of Medicine, Baltimore, Maryland.


The objective of the ProgStar studies is to determine the progression of disease in a large population of children and adults over at least a two year period, with a variety of potential measures.


  • To assess the yearly rate of progression of STGD using the growth or the development of atrophic lesions as measured by FAF imaging


  • To assess the yearly rate of progression of STGD using Spectral domain optical coherence tomography (SD-OCT) to measure the rates of retinal thinning and the loss of photoreceptors
  • To assess the yearly rate of loss of retinal sensitivity as measured by microperimetry (MP)
  • To assess the yearly rate of BCVA changes; in the prospective study, measurements will be based on measurements the Early Treatment Diabetic Retinopathy Study (ETDRS) protocol
  • To correlate the presence and progression of morphological abnormalities in FAF and SD-OCT images with visual function as measured by MP and VA
  • To perform exploratory analysis of factors associated with STGD progression, such as participant’s use of vitamin A supplementation and mutations in the ABCA4 gene

The study is sponsored by the Foundation Fighting Blindness, an organization dedicated to fund research that may lead to prevention, treatments, and cures for retinal degenerative diseases including STGD.