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Review Retinitis Pigmentosa Intravitreal Pharmacotherapy in the Treatment of Retinitis Pigmentosa-related Cystoid Macular Oedema Oya Donmez 1 and Ali Osman Saatci 2 1. TC Hitit University, Corum State Hospital, Corum, Turkey; 2. Dokuz Eylul University, Izmir, Turkey P eripheral visual field constriction and night blindness are the main features of retinitis pigmentosa (RP). However, central vision is often impaired dramatically even in the early stages of the disease when macular complications such as cystoid macular oedema (CME) occur. The pathogenesis of RP-related CME is still not well explained. Therefore, several treatment alternatives such as topical, oral and systemic pharmacotherapy; laser photocoagulation and even vitreoretinal surgery are employed by the clinicians. In this review, we summarise the clinical data on intravitreal pharmacotherapeutic agents and focus mainly on the steroids and anti-vascular endothelial growth factor agents. Keywords Aflibercept, intravitreal injection, macula, macular oedema, Ozurdex ® , ranibizumab, retinitis pigmentosa, steroid, vascular endothelial growth factor Disclosure: Oya Donmez and Ali Osman Saatci have nothing to disclose in relation to this article. No funding was received for the publication of this article. This study involves a review of the literature and did not involve any studies with human or animal subjects performed by any of the authors Authorship: All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship for this manuscript, take responsibility for the integrity of the work as a whole and have given final approval for the version to be published. Open Access: This article is published under the Creative Commons Attribution Noncommercial License, which permits any non-commercial use, distribution, adaptation, and reproduction provided the original author(s) and source are given appropriate credit. Received: 9 March 2017 Accepted: 20 April 2017 Citation: European Ophthalmic Review, 2017;11(1):55–8 Corresponding Author: Ali Osman Saatci, Mustafa Kemal Sahil Bulvari, No: 73, A Blok D:9, 35320 Narlidere/ Izmir, Turkey. E: Retinitis pigmentosa (RP) is an inherited retinal dystrophy characterised with the progressive loss of photoreceptors. 1,2 Night blindness together with the peripheral visual field loss is the most prominent clinical feature. 1,2 Central vision is relatively spared up to the later stages of the disease process. 3 However, when macular complications such as macular oedema arise, central vision is often dramatically impaired even in the early stages of the disease. The prevalence of cystoid macular oedema (CME) is reported to be 10–20% in the eyes of patients with RP. 4,5 The pathogenesis of RP-related CME is still unclear. Blood–retinal barrier (BRB) impairment, retinal pigment epithelium (RPE) pump disturbance, inflammation, autoimmunity and vitreoretinal interface changes may be among the potential causes of CME. 6–19 Fishman et al. 6 examined 15 RP patients with vitreous fluorophotometry. All patients showed abnormally high concentrations of fluorescein within the vitreous which was a sign of BRB abnormality. Larsen et al. 7 demonstrated that BRB leakage was markedly increased in six RP patients with the help of ocular spectrofluorophotometry. Vinores et al. 9 investigated the immunohistochemical staining for albumin on paraffin sections of 22 normal and 29 RP eyes. Electron microscopic immunocytochemical staining for albumin was performed on additional six normal and nine RP-affected eyes. Two-thirds of the eyes with RP demonstrated extravascular albumin in the inner portion of the posterior retina. This was evident even in the absence of CME but eyes with CME showed extensive BRB failure. Küchle et al. 10 analysed the BRB in patients with RP with a laser cell photometer in 56 eyes of 29 patients and found that aqueous flare values were higher in patients with RP than in controls; moreover, patients with RP-related CME had even higher values with a mean of 14.66 photon counts per millisecond compared with 9.65 for patients with RP but having no CME. RPE seems to lose polarised apical distribution in association with macular oedema and RP and cannot effectively pump out ions and fluid from the outer retina. 11 Autoimmune processes and/or inflammation can be deemed as the hallmark of RP-related CME. Spalton et al. 12 examined 25 patients with RP and all patients had an excessive number of vitreous cells; six even had exudates in the pre-equatorial fundus indistinguishable from pars planitis. They suggested that this might be a general response noticed in many types of tapetoretinal degeneration in reaction to actually degenerating photoreceptors or RPE. Furthermore, Heckenlively et al. 13 looked for the presence of antiretinal antibodies in a group of 30 consecutive patients with CME and RP, 30 consecutive patients with RP but without CME and 50 normal subjects. Twenty-seven (90%) of the patients with RP and CME had antiretinal protein antibody activity compared with three of 50 normal subjects (6%) and only four of 30 patients (13%) with RP but without coexistent CME. Yoshida et al. 14 studied the nature of inflammatory reaction in eyes of patients with RP. In 190 of 509 eyes (37.3%) with RP, ‘1+’ (5–9 cells per field) or more cells were observed in the TOU CH MED ICA L MEDIA 55