Retinitis Pigmentosa: Review of Current Treatment

Wang, Angeline L.; Knight, Darren; Vu, Thanh Thao T.; Mehta, Mitul C.

doi:10.1097/iio.0000000000000256

Cited by 59 publications

(44 citation statements)

References 83 publications

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“…Therefore, understanding mechanisms underlying retinal degeneration is a goal to both prevent and counteract blinding diseases. Among degenerative diseases of the retina, ischemic retinopathies are due to metabolic alterations of the retinal environment as in rubeosis iridis (RI), diabetic retinopathy (DR), retinopathy of prematurity (ROP) or age-related macular degeneration (AMD) [2,4] while retinal dystrophies are the consequence of genetic defects as in retinitis pigmentosa (RP) [5]. The first group of diseases is characterized by vasopermeability excess eventually leading to neovascularization, then resulting in retinal cell death (for Ref., see [6]).…”

Section: Degenerative Diseases Of the Retinamentioning

confidence: 99%

The uPAR System as a Potential Therapeutic Target in the Diseased Eye

Cammalleri

Monte

Pavone

et al. 2019

Cells

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Dysregulation of vascular networks is characteristic of eye diseases associated with retinal cell degeneration and visual loss. Visual impairment is also the consequence of photoreceptor degeneration in inherited eye diseases with a major inflammatory component, but without angiogenic profile. Among the pathways with high impact on vascular/degenerative diseases of the eye, a central role is played by a system formed by the ligand urokinase-type plasminogen activator (uPA) and its receptor uPAR. The uPAR system, although extensively investigated in tumors, still remains a key issue in vascular diseases of the eye and even less studied in inherited retinal pathologies such as retinitis pigmantosa (RP). Its spectrum of action has been extended far beyond a classical pro-angiogenic function and has emerged as a central actor in inflammation. Preclinical studies in more prevalent eye diseases characterized by neovascular formation, as in retinopathy of prematurity, wet macular degeneration and rubeosis iridis or vasopermeability excess as in diabetic retinopathy, suggest a critical role of increased uPAR signaling indicating the potentiality of its modulation to counteract neovessel formation and microvascular dysfunction. The additional observation that the uPAR system plays a major role in RP by limiting the inflammatory cascade triggered by rod degeneration rises further questions about its role in the diseased eye.

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Section: Degenerative Diseases Of the Retinamentioning

confidence: 99%

The uPAR System as a Potential Therapeutic Target in the Diseased Eye

Cammalleri

Monte

Pavone

et al. 2019

Cells

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“…However, several issues, such as numerous inheritance patterns, safety concerns, targeting, and low cargo capacity of the vector or necessity for an invasive administration route during pars plana vitrectomy, are problematic in RP patients. These methods presented acceptable safety levels but poor efficacy; thus, the idea of neuroprotective treatment in RP seems to be a promising direction for further research [7]. The neuroprotective effect was evaluated after an intraocular implant with retinal pigment epithelium (RPE) cells transfected with the human ciliary neurotrophic factor (CNTF) gene due to CNTF production.…”

Section: Introductionmentioning

confidence: 99%

Long-Term Effects of Adjuvant Intravitreal Treatment with Autologous Bone Marrow-Derived Lineage-Negative Cells in Retinitis Pigmentosa

Wiącek

Gosławski

Grabowicz

et al. 2021

Stem Cells International

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Background. Autologous bone marrow-derived lineage-negative (Lin−) cells present antiapoptotic and neuroprotective activity. The aim of the study was to evaluate the safety and efficacy of novel autologous Lin− cell therapy during a 12-month follow-up period. Methods. Intravitreal injection of Lin− cells in 30 eyes with retinitis pigmentosa (RP) was performed. The fellow eyes (FEs) were considered control eyes. Functional and morphological eye examinations were performed before and 1, 3, 6, 9, and 12 months after the injection. Results. Patients whose symptoms started less than 10 years ago gained 14 ± 10 letters, while those with a longer disease duration gained 2.86 ± 8.54 letters compared to baseline at the 12-month follow-up ( p = 0.021 ). There were significantly higher differences in response densities of P 1 -wave amplitudes in the first ring of multifocal ERGs in treated eyes than FE recordings in all follow-up points were detected. Accordingly, the mean deviation in 10-2 static perimetry improved significantly in the treated eyes compared with fellow eyes 12 months after the procedure. The QoL scores improved significantly and lasted until the 9-month visit. Conclusion. Lin− cell-based therapy is safe and effective, especially for a well-selected group of RP patients who still maintained good function of the foveal cones.

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“…Thus, the prognosis is usually quite heterogeneous. Acquired factors such as nutrition, smoking, anemia, pregnancy, as well as long-term exposure to ultraviolet and blue light also affect the course of the disease [2][3][4]. Autosomal dominant inheritance shows the slowest progression with an average annual loss of 5% photoreceptors [20,21].…”

Section: Figures 125mentioning

confidence: 99%

“…The initial symptom of the disease is usually night blindness (nyctalopia) beginning in childhood or adolescence. Narrowing of the visual field and legal blindness develops as the disease progresses [2][3][4]. If low grade inflammation is added, then the disease is complicated by cataracts, an epiretinal membrane, and macular edema [5].…”

Section: Introductionmentioning

confidence: 99%

Management of Retinitis Pigmentosa via Platelet Rich Plasma or Combination with Electromagnetic Stimulation: Retrospective Analysis of One-year Results

Arslan

Özmert

2020

Preprint

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Purpose To investigate whether the natural progression rate of retinitis pigmentosa can be decreased with subtenon autologous platelet rich plasma application alone or combination with retinal electromagnetic stimulation.Material and methods The study includes retrospective analysis of 60 retinitis pigmentosa patients. Patients constitute 3 groups with similar demographic characteristics. The combined management group consists of 20 retinitis pigmentosa patients (40 eyes) who received combined retinal electromagnetic stimulation and subtenon platelet rich plasma as Group1; The subtenon platelet rich plasma-only group consisted of 20 retinitis pigmentosa patients (40 eyes) as Group2; The natural course (control) group consists of 20 retinitis pigmentosa patients (40 eyes) who did not receive any treatment were classified as Group3. Horizontal and vertical ellipsoid zone width, fundus perimetry deviation index and best corrected visual acuity changes were compared within and between groups after one year follow up period.Results Horizontal ellipsoid zone percentage changes were detected +1 % in Group1, -2.85% in Group2, -9.36% in Group3 (Δp 1>2>3). Vertical ellipsoid zone percentage changes were detected +0.34 % in Group1, -3.05% in Group2, -9.09% in Group3 (Δp 1>2>3). Fundus perimetry deviation index percentage changes were detected +0.05% in Group1, -2.68% in Group2 and -8.78% in Group3 (Δp 1>2>3). Conclusion Platelet-rich plasma is a good source of growth factors, but its half-life is 4-6 months. Subtenon autologous platelet rich plasma might more effectively slow down photoreceptor loss when repeated as booster injections and combined with retinal electromagnetic stimulation.

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Retinitis Pigmentosa: Review of Current Treatment

Cited by 59 publications

References 83 publications

The uPAR System as a Potential Therapeutic Target in the Diseased Eye

The uPAR System as a Potential Therapeutic Target in the Diseased Eye

Long-Term Effects of Adjuvant Intravitreal Treatment with Autologous Bone Marrow-Derived Lineage-Negative Cells in Retinitis Pigmentosa

Management of Retinitis Pigmentosa via Platelet Rich Plasma or Combination with Electromagnetic Stimulation: Retrospective Analysis of One-year Results

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