Association between retinitis pigmentosa and an increased risk of primary angle closure glaucoma: A population-based cohort study

Abstract

Background Retinitis pigmentosa (RP) is the most frequent retinal hereditary dystrophy and result in blindness if progresses. Several case reports have revealed the possible association between RP and primary angle-closure glaucoma (PACG). We conducted a population-based study to explore whether RP significantly increased the risk of PACG development. Methods Using the Taiwan National Health Insurance Research Database, we enrolled patients with RP into the RP group from 2001 to 2013 and included a comparison group of 1:4 age- and sex-matched individuals without RP. We performed a Cox regression analysis to estimate the crude and adjusted hazard ratios (HRs) of RP for PACG after adjustment for hypertension, diabetes, hyperlipidaemia, chronic kidney disease, and lens subluxation. Results We enrolled 6223 subjects with RP and 24892 subjects for comparison. The mean age of the cohort was 49.0 ± 18.1 years. The RP group had significantly higher percentages of diabetes mellitus, hypertension, and hyperlipidaemia. The cumulative incidence of PACG in patients with RP was 1.61%, which was significantly higher than that in the comparison group (0.81%, p < 0.0001). According to the univariate Cox regression analysis, the hazard of PACG development was significantly greater in the RP group, with an unadjusted HR of 2.09 (95% confidence interval [CI], 1.64–2.65). The increased risk persisted after adjusting for confounders (adjusted HR = 2.18; 95% CI, 1.76–2.72). Conclusion This nationwide population-based cohort study showed that people with RP are at a significantly greater risk of developing PACG than individuals without RP.

Retinitis Pigmentosa: Current Clinical Management and Emerging Therapies

“…A common form of glaucoma found in RP is primary angle-closure glaucoma (PACG), with prevalence rates between 1.0% and 2.3% [ 262 , 263 , 264 ]. Previous studies have shown that the association between RP and PACG are related to nanophthalmos, short axial length, cataract and lens subluxation [ 263 ]. Anatomically, patients with a short axial length and/or cataract have a relatively shallow anterior chamber more prone to occlusion.…”

Commentary: Visual impairment due to retinitis pigmentosa in India

“…While the association of primary open-angle glaucoma in RP is known, people with RP are also at a significantly higher risk of developing primary angle-closure glaucomas. [ 5 , 6 ] One possible explanation is the underlying genetic factors leading to dystrophic changes both in the trabecular meshwork and retina. It has been noted that these patients may have a tendency toward accelerated lens growth, which can contribute to a higher risk of angle-closure glaucomas.…”

“…[ 7 ] There is also a higher risk of lens subluxation in patients with RP, which can further aggravate narrow angles. [ 5 ]…”