Abstract
Retinitis pigmentosa is a clinical and genetic group of inherited retinal disorders characterized by alterations of photoreceptors and retinal pigment epithelium leading to a progressive concentric visual field restriction, which may bring about severe central vision impairment. Haemodynamic studies in patients with retinitis pigmentosa have demonstrated ocular blood flow abnormalities both in retina-choroidal and in retroocular vascular system. Moreover, several investigations have studied the augmentation of endothelin-1 plasma levels systemically in the body and locally in the eye. This might account for vasoconstriction and ischemia, typical in vascular dysregulation syndrome, which can be considered an important factor of reduction of the ocular blood flow in subjects affected by retinitis pigmentosa.
Highlights
Recent studies have observed the reduced blood flow in both ocular and peripheral districts in patients suffering from retinitis pigmentosa (RP) [1]
In the presence of excessive vasoconstriction and/or altered vasodilation, the overall condition is described as vascular dysregulation and it is often accompanied by a barrier dysfunction
Big efforts are still to be made to assess a fine correlation between genetic mutations and clinical manifestations in the RP
Summary
Recent studies have observed the reduced blood flow in both ocular and peripheral districts in patients suffering from retinitis pigmentosa (RP) [1]. Using colour Doppler imaging, Cellini and coworkers demonstrated reduced peak systolic velocities in both ophthalmic arteries and posterior ciliary arteries [3, 4] They observed a certain augmentation of endothelin-1 (ET-1) plasma levels in patients with earlystage RP, even though some investigators took issue with this observation [6,7,8]. Disturbed autoregulation of ocular perfusion provokes an irregular blood flow, which means an unstable retinal blood supply, and a sort of attenuation of retinal vessels with reduced neurovascular coupling [14, 15]. Both free radicals and oxidative stress substantially increase. Even if the exact pathophysiological mechanism is still unknown, the dysfunction of both autonomic nervous system and endothelial cells is currently investigated
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