Locus-Level Changes in Macular Sensitivity in Patients with Retinitis Pigmentosa Treated with Oral N-acetylcysteine

PURPOSE: To identify characteristics of loci associated with locus-level sensitivity loss or improvement during treatment with N-acetylcysteine (NAC) in retinitis pigmentosa (RP). DESIGN: Retrospective analysis of prospectively collected data in the FIGHT RP clinical trial. METHODS: Patients (n = 30) were treated with 600, 1,200, or 1,800 mg of NAC twice daily for 3 months and then 3 times/day for 3 months. Microperimetry locus-level changes between baseline and month 6 were correlated with baseline characteristics of loci using regression models. The main outcome measurement was locus-level sensitivity change >= 6 dB. RESULTS: Baseline mean sensitivity (3,468 loci; 51 evaluable eyes) was 7.7 dB and for foveal, parafoveal, and perifoveal loci were 20.2, 11.8, and 5.8 dB. During treatment, 287 loci (8.28%) increased >= 6 dB, and 119 of 1,613 loci with baseline sensitivity >= 6 dB decreased >= 6 dB (7.38%). A higher dose of NAC was associated with lower likelihood of sensitivity loss >= 6 dB (P = .033). Loci with low baseline sensitivity were more likely to decrease >= 6 dB (P = .034) but also more likely to increase >= 6 dB (P<.001). Foveal versus perifoveal loci (P<.001) and superior versus inferior loci (P = .005) were more likely to increase >= 6 dB. CONCLUSIONS: Higher doses of NAC reduced risk of macular loci sensitivity loss in RP. Greater sensitivity depression reversibility in the fovea during treatment suggests that high foveal cone density protects cones from irreversible loss of function in RP making them more likely to show improved function during NAC treatment. ((C) 2020 Elsevier Inc. All rights reserved.)

Automated summary

In patients with RP, higher doses of NAC reduced the risk of macular locus sensitivity loss, while the higher resolution of sensitivity depression in the retina during treatment suggests that high foveal cone density protects cones from irreversible loss of function in RP, making them more likely to show improved function during NAC treatment.