Cataractogenesis In Retinitis Pigmentosa

Bellelli, Andrea; Santori, M; Pannarale, Luigi; Forte, Renato; Iannaccone, Alessandro; Grenga, Roberto

doi:10.1007/978-1-4615-1897-6_45

Cited by 3 publications

(4 citation statements)

References 15 publications

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“…The early investigations into the role of the immune system in RP pathogenesis and progression led us to investigate the therapeutic potential of an IMT agent available in the 1990s, thymopentin ( Rispoli et al, 1990 ; Rispoli et al, 1991 ; Vingolo et al, 1993a ; Vingolo et al, 1993b ; Iannaccone et al, 1994a ). Administered i.v., thymopentin reduces immune system activation.…”

Section: Introductionmentioning

confidence: 99%

“…Administered i.v., thymopentin reduces immune system activation. In brief, in an open-label, prospective, pilot trial comparing automated visual field (VF) sensitivity and retinal function by virtue of the mixed full-field flash electroretinogram (ffERG) b-wave amplitudes to a historical natural history control group ( Berson et al, 1985 ), serial intravenous thymopentin administration improved both parameters at 18 and 36 mos ( Rispoli et al, 1990 ; Rispoli et al, 1991 ; Vingolo et al, 1993a ; Vingolo et al, 1993b ; Iannaccone et al, 1994a ) vs. both declining progressively in the historical control group ( Berson et al, 1985 ). These results supported the hypothesis that immune system activation is a contributing factor to IRD pathobiology and progression.…”

Section: Introductionmentioning

confidence: 99%

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The double-edged sword of inflammation in inherited retinal degenerations: Clinical and preclinical evidence for mechanistically and prognostically impactful but treatable complications

Sarıcı

Vyas

Iannaccone

2023

Front. Cell Dev. Biol.

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We present retrospective data from our clinical research efforts of the past several years alongside a review of past and current clinical and preclinical data independently by several investigators supporting our clinical evidence for the importance of inflammation in inherited retinal degenerations (IRDs). We show how inflammation is a complicating factor in IRDs but, if recognized and managed, also a great opportunity to mitigate disease severity immediately, improve patient prognosis and quality of life, extend the treatment windows for gene-specific and agnostic therapeutic approaches, mitigate the impact of inflammatory complications on the accurate estimate of vision changes in IRD natural history studies, improve the chances of safer outcomes following cataract surgery, and potentially reduce the likelihood of inflammatory adverse events and augment the efficacy of viral vector-based treatment approaches to IRDs. Manuscript contribution to the field. Inflammation has been suspected to be at play in IRDs since the beginning of the 1900s and became a research focus through the early 1990s but was then largely abandoned in favor of genetic-focused research. Thanks to regained cognizance, better research tools, and a more holistic approach to IRDs, the recent reappraisal of the role of inflammation in IRDs has brought back to the surface its importance. A potential confounder in natural history studies and a limiting factor in clinical trials if not accounted for, inflammation can be managed and often offers an opportunity for immediately improved prognosis and outcomes for IRD patients. We present our retrospective clinical evidence for connections with a measurable secondary autoimmune component that can develop in IRDs and contribute to vision loss but is at least in part treatable. We also present ample lines of evidence from the literature corroborating our clinical observations at the preclinical level.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The double-edged sword of inflammation in inherited retinal degenerations: Clinical and preclinical evidence for mechanistically and prognostically impactful but treatable complications

Sarıcı

Vyas

Iannaccone

2023

Front. Cell Dev. Biol.

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“…Furthermore, our findings suggest that antioxidant supplements may improve morphological damage (CMT). Indeed, as reported in the literature, ROS promotes breakdown of the blood-retinal barrier and alters retinal blood flow by modulating the production of vasoactive factors, also upregulating the retinal expression of VEGF and adhesion molecules as ICAM-1 [31][32][33][34][35]. This cascade of molecular events causes leakage of fluids into the surrounding tissue, which initially accumulate within Müller cells.…”

mentioning

confidence: 80%

Retinitis Pigmentosa (RP): The Role of Oxidative Stress in the Degenerative Process Progression

et al. 2022

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Purpose: Retinitis Pigmentosa is a term that includes a group of inherited bilateral and progressive retinal degenerations, with the involvement of rod photoreceptors, which frequently leads to blindness; oxidative stress may be involved in the degeneration progression as proposed by several recent studies. The goal of this study is to evaluate whether circulating free radicals taken from capillary blood are related to one of the most important features of Retinitis pigmentosa that can affect frequently patients: cystoid macular oedema (CME). Materials: A total of 186 patients with Retinitis Pigmentosa (range: 25–69 years) were enrolled; all patients completed an ophthalmologic examination and SD-OCT at baseline and were divided into three subgroups according to the SD-OCT features. ROS blood levels were determined using FORT with monitoring of free oxygen radicals. Results: Test levels of free oxygen radicals were significantly increased, almost twice, in RP patients showing cystoid macular oedema and significantly increased compared to the control group. (p < 0.001). Discussion: Our findings suggest that oxidative stress may speed cone photoreceptors’ morphological damage (CMT); because long lasting oxidative stress in the RP may cause oxidative damage, with animal models of RP suggesting this is a micromolecular mechanism of photoreceptors’ (cone) death, it can be similar to cone damage in human RP eyes. The limitations of this paper are the relatively small sample, the horizontal design of the study, and the lack of data about the levels of ROS in the vitreous body.

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“…48 Vingolo et al reported a significantly lower probability of postoperative PCO with the hydrophobic acrylic IOLs with a sharp-edged design in patients with RP compared with other types of IOL implants. 49…”

Section: Methodsmentioning

confidence: 99%