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Ruben Jauregui, Vitor K. L. Takahashi, Karen Sophia Park, Julia Takiuti, Stephen H Tsang; Multimodal structural disease progression of retinitis pigmentosa according to mode of inheritance. Invest. Ophthalmol. Vis. Sci. 2019;60(9):4528. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
To analyze disease progression according to mode of inheritance in patients with retinitis pigmentosa (RP) by quantifying the progressive decrease of the ellipsoid zone (EZ) line width on spectral domain optical coherence tomography (SD-OCT) scans and the dimensions of the hyperautofluorescent ring on short-wave fundus autofluorescence (SW-FAF).
This is a retrospective cohort study on 115 eyes from 115 patients with a diagonosis of RP. Measurements of the EZ line width on SD-OCT scans and horizontal diameter and ring area on SW-FAF imaging were performed by two independent graders. SD-OCT and SW-FAF imaging were performed on each patient on two separate clinic visits, at least 1 year apart.
The average follow-up time was 3.2 ± 1.9 years. EZ line width declined at a rate of -121 ± 7 µm per year (P < 0.001). The horizontal diameter and ring area declined at a rate of -133 ± 8 µm (P < 0.001) and -0.5 ± 0.04 mm2 per year (P < 0.001), respectively. Disease progression was found to be slowest for autosomal dominant RP and fastest for X-linked RP (XLRP), with autosomal recessive RP progression rates between those of adRP and XLRP. Ring area progression rates were significantly different when comparing adRP and XLRP, but not when comparing arRP with either adRP or XLRP.
Using EZ line width and the horizontal diameter and area of the hyperautofluorescent ring as parameters of disease progression, we confirm that adRP is the slowest progressing form of RP while XLRP is the fastest. The rates for these parameters can serve as benchmarks for investigators of future clinical trials for RP and its different modes of inheritance.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.
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