Müller PL, Gliem M, McGuinnes M, et al. - In this cross-sectional study with age-matched healthy controls, researchers examined lipofuscin-related quantitative autofluorescence measures and their connection with demographic characteristics, retinal structure, retinal function and genotype in ABCA4-related retinopathy (Stargardt disease 1). In total, 77 patients with ABCA4-related retinopathy and 110 controls had quantitative fundus autofluorescence (qAF) imaging using a confocal scanning laser ophthalmoscope equipped with an internal fluorescent reference to measure lipofuscin accumulation. According to findings, most patients (76.6%) had qAF levels above the 95% prediction interval of the age-related control group, with the best disparity between cases and controls in younger patients. Reduced discrimination on the basis of qAF measures was correlated with very mild disease, a more advanced disease with dark flecks, or older age due to physiological age-related increase in qAF and a ceiling effect in patients. Accumulation of lipofuscin is dependent on the severity of variants of ABCA4, precedes other structural changes, and may remain without clinically significant retinal function influence.