June 2015
Volume 56, Issue 7
Free
ARVO Annual Meeting Abstract  |   June 2015
Fundus Autofluorescence Characteristics in the Natural History of the Progression of Atrophy secondary to Stargardt Disease (PROGSTAR) Study
Author Affiliations & Notes
  • Syed Mahmood Shah
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD
  • Xiangrong Kong
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD
  • Alexander Ho
    Doheny Eye Institute, David Geffen School of Medicine, University of California-Los Angeles, Los Angeles, CA
    Doheny Image Reading Center, Doheny Eye Institute, Los Angeles, CA
  • Srinivas R Sadda
    Doheny Eye Institute, David Geffen School of Medicine, University of California-Los Angeles, Los Angeles, CA
    Doheny Image Reading Center, Doheny Eye Institute, Los Angeles, CA
  • Beatriz E Munoz
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD
  • Rupert Wolfgang Strauss
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD
    Department of Ophthalmology, Medical University Graz, Graz, Austria
  • Mohamed Ibrahim
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD
  • Yulia Wolfson
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD
  • Sheila K West
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD
  • Hendrik P Scholl
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD
  • Footnotes
    Commercial Relationships Syed Mahmood Shah, None; Xiangrong Kong, None; Alexander Ho, None; Srinivas Sadda, Carl Zeiss Meditec (C), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (F), Carl Zeiss Meditec (R), Optos (C), Optos (F), Optos (R); Beatriz Munoz, None; Rupert Strauss, None; Mohamed Ibrahim, None; Yulia Wolfson, None; Sheila West, None; Hendrik Scholl, QLT Inc. (C), QLT Inc. (F), Sanofi-Fovea Pharmaceuticals (C), Vision Medicines, Inc (C)
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science June 2015, Vol.56, 1652. doi:
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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, ; 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.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract
 
Purpose
 

To characterize fundus autofluorescence (AF) of patients with Stargardt disease (STGD) as part of the PROGSTAR study.

 
Methods
 

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.

 
Results
 

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).

 
Conclusions
 

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).  

 
Table 1: AF characteristics as graded by RegionFinder™.
 
Table 1: AF characteristics as graded by RegionFinder™.
 
 
Table 2: DDAF classification.
 
Table 2: DDAF classification.

 
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