title: Differential Disease Progression in Atrophic Age-Related Macular Degeneration and Late-Onset Stargardt Disease.
abstract: To compare the disease course of retinal pigment epithelium (RPE) atrophy secondary to age-related macula degeneratio (AMD) and late-onset Stargardt disease (STGD1).
  
  
  
  Patients were examined longitudinally by fundus autofluorescence, near-infrared reflectance imaging, and best-corrected visual acuity (BCVA). Areas of RPE atrophy were quantified using semi-automated software, and the status of the fovea was evaluated based on autofluorescence and near-infrared reflectance images. Mixed-effects models were used to compare atrophy progression rates. BCVA loss and loss of foveal integrity were analyzed using Turnbull's estimator.
  
  
  
  A total of 151 patients (226 eyes) with RPE atrophy secondary to AMD and 38 patients (66 eyes) with RPE atrophy secondary to late-onset STGD1 were examined for a median time of 2.3 years (interquartile range, 2.7). Mean baseline age was 74.2 years (SD, 7.6) in AMD and 63.4 (SD, 9.9) in late-onset STGD1 (P = 1.1 × 10-7). Square root atrophy progression was significantly faster in AMD when compared with late-onset STGD1 (0.28 mm/year [SE, 0.01] vs. 0.23 [SE, 0.03]; P = 0.030). In late-onset STGD1, the median survival of the fovea was significantly longer when compared with eyes with AMD (8.60 vs. 3.35 years; P = 0.005) with a trend to a later BCVA loss of ≥3 lines (5.97 vs. 4.37 years; P = 0.382).
  
  
  
  These natural history data indicate differential disease progression in AMD versus late-onset STGD1. The results underline the relevance of refined phenotyping in elderly patients presenting with RPE atrophy in regard to prognosis and design of interventional trials.
authors.affiliation: Department of Ophthalmology, University of Bonn, Bonn, Germany.
authors.forename: Moritz
authors.initials: M
authors.lastname: Lindner
authors.affiliation: Department of Ophthalmology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Stanley
authors.initials: S
authors.lastname: Lambertus
authors.affiliation: Department of Ophthalmology, University of Bonn, Bonn, Germany.
authors.forename: Matthias M
authors.initials: MM
authors.lastname: Mauschitz
authors.affiliation: Department of Ophthalmology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Nathalie M
authors.initials: NM
authors.lastname: Bax
authors.affiliation: Department of Ophthalmology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Eveline
authors.initials: E
authors.lastname: Kersten
authors.affiliation: Department of Ophthalmology, University of Bonn, Bonn, Germany.
authors.forename: Anna
authors.initials: A
authors.lastname: Lüning
authors.affiliation: Institute for Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Nadal
authors.affiliation: Department of Ophthalmology, University of Bonn, Bonn, Germany.
authors.forename: Steffen
authors.initials: S
authors.lastname: Schmitz-Valckenberg
authors.affiliation: Institute for Medical Biometry, Informatics and Epidemiology, University of Bonn, Bonn, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Schmid
authors.affiliation: Department of Ophthalmology, University of Bonn, Bonn, Germany.
authors.forename: Frank G
authors.initials: FG
authors.lastname: Holz
authors.affiliation: Department of Ophthalmology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Carel B
authors.initials: CB
authors.lastname: Hoyng
authors.affiliation: Department of Ophthalmology, University of Bonn, Bonn, Germany.
authors.forename: Monika
authors.initials: M
authors.lastname: Fleckenstein
mesh_terms: D000368:Aged; D018450:Disease Progression; D004596:Electroretinography; D005260:Female; D005451:Fluorescein Angiography / Q000379:methods*; D005500:Follow-Up Studies; D005654:Fundus Oculi; D006801:Humans; D008266:Macula Lutea / Q000473:pathology* / Q000503:physiopathology; D008268:Macular Degeneration / Q000151:congenital* / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D055213:Retinal Pigment Epithelium / Q000473:pathology*; D012189:Retrospective Studies; D000080362:Stargardt Disease; D041623:Tomography, Optical Coherence; D014792:Visual Acuity