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Abstract
We analyze disease progression in retinitis pigmentosa (RP) according to mode of inheritance by quantifying the progressive decrease of the ellipsoid zone (EZ) line width on spectral domain optical coherence tomography (SD-OCT) and of the dimensions of the hyperautofluorescent ring on short-wave fundus autofluorescence (SW-FAF). In this retrospective study of 96 patients, average follow-up time was 3.2 ± 1.9 years. EZ line width declined at a rate of −123 ± 8 µm per year, while the horizontal diameter and ring area declined at rates of −131 ± 9 µm and −0.5 ± 0.05 mm2 per year, respectively. Disease progression was found to be slowest for autosomal dominant RP and fastest for X-linked RP, with autosomal recessive RP progression rates between those of adRP and XLRP. EZ line width and ring diameter rates of disease progression were significantly different between each mode of inheritance. By using EZ line width and horizontal diameter as parameters of disease progression, our results confirm that adRP is the slowest progressing form of RP while XLRP is the fastest. Furthermore, the reported rates can serve as benchmarks for investigators of future clinical trials for RP and its different modes of inheritance.
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1 Department of Ophthalmology, New York-Presbyterian Hospital, New York, NY, USA; Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory, New York, NY, USA; Weill Cornell Medical College, New York, NY, USA
2 Department of Ophthalmology, New York-Presbyterian Hospital, New York, NY, USA; Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory, New York, NY, USA; Department of Ophthalmology, Federal University of São Paulo, São Paulo, Brazil
3 Department of Ophthalmology, New York-Presbyterian Hospital, New York, NY, USA; Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory, New York, NY, USA
4 Department of Ophthalmology, New York-Presbyterian Hospital, New York, NY, USA; Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory, New York, NY, USA; Division of Ophthalmology, University of São Paulo Medical School, São Paulo, Brazil
5 Department of Ophthalmology, New York-Presbyterian Hospital, New York, NY, USA; Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory, New York, NY, USA; Department of Ophthalmology, Federal University of São Paulo, São Paulo, Brazil; Departament of Ophthalmology, Empresa Brasileira de Servicos Hospitalares (EBSERH) - Hospital das Clinicas de Pernambuco (HCPE), Federal University of Pernambuco (UFPE), Recife, Brazil
6 Department of Ophthalmology, New York-Presbyterian Hospital, New York, NY, USA; Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory, New York, NY, USA; Department of Pathology & Cell Biology, Stem Cell Initiative (CSCI), Institute of Human Nutrition, College of Physicians and Surgeons, Columbia University, New York, NY, USA