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Publications

Journal Article Ppublish

PubMed: 41773771 DOI: 10.1167/iovs.67.3.6

Longitudinal Changes of Fundus Autofluorescence and Correlation With Visual Acuity in ABCA4-Associated Stargardt Disease.

Invest Ophthalmol Vis Sci 67(3):6

Authors: Folahan Ibukun, Rupert W Strauss, Zainab Rustam, Muhammad Jehanzeb Khan, Artur V Cideciyan, David G Birch, Alexander Ho, Janet S Sunness, Isabelle S Audo, Michel Michaelides, Eberhart Zrenner, Elias I Traboulsi, Paul Bernstein, SriniVas Sadda, Michael S Ip, Sheila West, Xiangrong Kong

Affiliations

  • Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, United States.
  • Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, United Kingdom.
  • Department of Ophthalmology, Kepler University Clinic, Linz, Linz, Austria.
  • Department of Ophthalmology, Medical University of Graz, Graz, Austria.
  • Department of Ophthalmology, University of Basel, Basel, Switzerland.
  • Scheie Eye Institute, University of Pennsylvania, Philadelphia, Pennsylvania, United States.
  • Retina Foundation of the Southwest, Dallas, Texas, United States.
  • Doheny Image Reading Center, Doheny Eye Institute, Pasadena, California, United States.
  • Hoover Low Vision Rehabilitation Services, Greater Baltimore Medical Center, Baltimore, Maryland, United States.
  • Sorbonne Université, INSERM, CNRS, Institut de la Vision; CHNO des Quinze-Vingts, Centre de Référence Maladies Rares REFERET and INSERM-DGOS CIC1423, Paris, France.
  • Center for Ophthalmology, Institute for Ophthalmic Research, University of Tübingen, Germany.
  • Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio, United States.
  • Moran Eye Center, University of Utah, Salt Lake City, UT, United States.
  • Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, United States. Department of Biostatistics, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, United States. Department of Epidemiology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, United States. Department of Health Behavior and Society, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland, United States.

Abstract

PURPOSE

To assess cross-sectional and longitudinal relationships between fundus autofluorescence (FAF) and visual acuity (VA) in ABCA4-associated Stargardt disease (STGD1).

METHODS

Secondary analysis used data from 301 STGD1 participants (566 study eyes) enrolled in the international ProgStar studies. The study reading center graded the FAF images qualitatively and quantitatively, including areas of atrophy reflected as definitely decreased autofluorescence (DDAF) and questionably decreased autofluorescence (QDAF). Linear models estimated cross-sectional and longitudinal associations between the growth of DDAF area and total DAF area (i.e., sum of DDAF and QDAF areas) and change in VA.

RESULTS

At the first visit, the median age was 30 years (range, 7-69); 55% were women. Mean ± SD VA was 0.73 ± 0.36 logMAR; mean total DAF area was 3.4 ± 3.8 mm2; and 42% of eyes had no DDAF. Cross-sectionally, increasing age and duration of symptoms, younger age of symptom onset, and larger QDAF area were significantly associated with worse VA (P < 0.05). Mean follow-up was 3.3 years (range, 0.5-10.3). There was no statistically significant relationship between growth of total DAF or DDAF areas and change in VA (P > 0.05).

CONCLUSIONS

Characteristics other than DAF area (e.g., flecks presence) may influence VA. Some patients remained DDAF free longitudinally and thus would be excluded in STGD1 trials that use DDAF as the primary endpoint. The cross-sectional relationship between DAF and VA confirmed the association of VA with retinal pigment epithelium (RPE) structure. The lack of a significant association between growth in DAF areas with change in VA over follow-up points to challenges resulting from slow progression and large VA variability. Exploring outcome measures from other modalities is warranted for designing STGD1 trials.

MeSH

Eye, Growth, Stargardt Disease, Ophthalmology, Visual Acuity