ProgStar: Prognosis for VA loss at 1 year for Stargardt

Seattle-Studies estimating the rate of visual acuity loss in patients with Stargardt disease have been based on retrospective chart reviews. The Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) studies are hoping to change that.

Speaking at ARVO 2016, Xiangrong Kong, PhD, explained that one issue with retrospective reviews is that data quality may be limited due to the inconsistency in testing procedures.

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Dr. Kong, of the Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, and colleagues used data from the prospective arm of the ProgStar study to estimate VA loss and to determine what, if any, associations could be made with demographic and clinical factors.

A total of 259 patients with confirmed Stargardt disease were enrolled from nine sites, followed for 6 and 12 months. Best-corrected visual acuity (BCVA) was measured with an Early Treatment of Diabetic Retinopathy Study chart.

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“Overall, the 1-year change in VA was minimal, but 12% of eyes did lose at least 1 line of vision,” Dr. Kong said. “Eyes with an initial good VA at baseline (20/25 or better) had significant VA loss over the course of the trial, declining at a rate of 0.04 logMAR.”

The median patient age was 31 years, with females comprising the majority, she added.

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The median age of onset was 19 years old, and median duration since symptom onset was 9 years. Of the patients, 14% (n = 35) were former smokers and 11% (n = 29) were current smokers.

Associated characteristics

Baseline BCVA was 0.78 logMAR.

Several characteristics were associated with a worse VA:

• retinal pigment epithelial pigmentation (0.1 logMAR worse);

• flecks outside the arcades (0.18 logMAR worse);

• juvenile symptom onset (0.22 logMAR worse), and

• longer symptom duration.

The risk of VA loss was highest (19%) in eyes with an initial VA of 20/70 or better.

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Dr. Kong noted the risk of VA loss was not associated with the presence of flecks outside the arcades, RPE pigmentation, smoking, age at symptom onset, or symptom duration.

Sponsored by the Foundation Fighting Blindness, the ProgStar studies comprise retrospective and prospective longitudinal observational studies.

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The retrospective study includes clinical examination findings and images collected between 2008 (and earlier) and 2014 and progression will be retrospectively evaluated, whereas the prospective study consists of a 24-month observational period from 2013/2014 for 2 years, with one visit every 6 months.

Dr. Kong has no financial disclosures to report.