Central and peripheral vision loss in inherited retinal diseases differentially affects quality of life

Abstract

Purpose : Inherited retinal diseases (IRDs) can initially affect central or peripheral vision and have variable impacts on quality of life (QoL). This prospective cross-sectional study evaluated the associations of both visual acuity (VA) and the type of vision loss (central vs. peripheral) with QoL in IRDs.

Methods : Adults with non-syndromic IRDs and VA better than 1.0 LogMAR were included. IRD phenotypes were classified into those initially affecting peripheral vision (retinitis pigmentosa and choroideremia) or central vision (macular and cone/cone-rod dystrophies). Binocular visual field (BVF) area was quantified from a composite of visual fields obtained from both eyes using Goldmann perimetry (V4e isopter). Vision-specific QoL was assessed using the 28-item Impact of Vision Impairment (IVI) and 6-item Vision and Quality of Life (VisQoL) questionnaires. An IVI score (mean of all item scores, range: 0-3) and weighted predicted VisQoL utility score was calculated (range: 0-1) for each person. Higher scores indicate better QoL in both questionnaires. Multivariable linear regression was used to evaluate if age, gender, best-eye VA, and IRD phenotype were associated with IVI and VisQoL scores.

Results : Of 70 included participants (mean age 44±20 years, 61% male), 46 (66%) had IRDs initially affecting peripheral vision and 24 had IRDs initially affecting central vision. Compared to those with central IRDs, participants with peripheral IRDs had better VA (Median [IQR]: central: 0.64 [0.37-0.88] vs. peripheral: 0.20 [0.08-0.44] LogMAR; p=0.001) but a smaller BVF area (central: 13865 [10723-15822] vs. peripheral: 5650 [422- 11337] degree²; p<0.001). Lower IVI scores were associated with worse VA (β=-0.46; p<0.001) and peripheral IRD phenotypes (β=-0.29; p=0.03. Model R²=0.13; p=0.01). Similarly, lower VisQoL scores were associated with worse VA (β=-0.34, p=0.01) and peripheral IRD phenotypes (β=-0.30, p=0.02. Model R²=0.08; p=0.06). Neither IVI or VisQoL predicted utility scores were associated with age or gender.

Conclusions : Both VA and the primary location of vision loss are associated with vision-specific QoL in IRDs. IRD-specific patient-reported measures may be better for distinguishing phenotype-specific symptoms in evaluating the impact of vision loss on QoL in IRDs.

This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.