The Pangere Center for Inherited Retinal Diseases, The Chicago Lighthouse, Chicago, Illinois; and.
Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois.
Retina. 2020 Aug;40(8):1630-1633. doi: 10.1097/IAE.0000000000002649.
Retinitis pigmentosa (RP) is an inherited pigmentary retinal dystrophy where patients experience poor peripheral, night, and eventually central vision. There are statements in the literature which suggest visual acuity loss can progress to total blindness in these patients. This study sought to examine these statements by performing a retrospective analysis of the visual acuity measured in a large cohort of RP patients.
The charts of 1,095 RP patients were reviewed in this retrospective cross-sectional analysis. They included all of the RP patients examined by one of the authors (G.A.F.). Patients with sector RP or a delimited form of this disease were not included. The review was focused on the analysis of patients with 20/200 or worse vision in the better-seeing eye (N = 215).
We determined that 0.46% of the enrolled patients progressed to no light perception in each eye. Ninety-two percent of the 1,095 patients examined were able to read a visual acuity chart. There were 6.8% who saw only hand motion, count fingers, or light perception.
No light perception was measured in only 0.46% of patients. Thus, only a very small number of the RP patients in our cohort progressed to total blindness.
色素性视网膜炎（RP）是一种遗传性色素性视网膜营养不良，患者会出现周边视力差、夜间视力差，最终出现中心视力差。有文献中的说法表明，这些患者的视力丧失可能会导致完全失明。本研究通过对大量 RP 患者的视力进行回顾性分析，旨在检验这些说法。
在这项回顾性横断面分析中，对 1095 名 RP 患者的图表进行了回顾。他们包括作者之一（G.A.F.）检查的所有 RP 患者。不包括扇形 RP 或这种疾病的限定形式。审查的重点是分析较好眼视力为 20/200 或更差的患者（N=215）。
我们确定，在每只眼中，有 0.46%的入组患者进展为无光感。在检查的 1095 名患者中，有 92%的患者能够阅读视力表。有 6.8%的患者只能看到手动运动、数手指或光感。
只有 0.46%的患者测量到无光感。因此，我们队列中的 RP 患者只有极少数进展为完全失明。
