Long-term efficacy and safety of anti-VEGF therapy in retinitis pigmentosa: a case report

Abstract

BackgroundRetinitis pigmentosa (RP), a neurodegenerative disease, is occasionally accompanied by choroidal neovascularization (CNV) and cystoid macular oedema. It is presently treated with repeated intravitreal injections of anti-vascular endothelial growth factor (VEGF) agents. However, there are concerns regarding long-term inhibition of VEGF by the use of these agents, especially in cases involving neurodegenerative diseases, since VEGFs have a neuroprotective effect. Currently, there are no reports on the long-term safety of anti-VEGF therapy in patients with RP.Case presentationIn this report, we describe the case of a 56-year-old female patient with CNV associated with RP who was treated with anti-VEGF therapy for 8 years. She had autosomal dominant RP with a heterozygous PRPH2 mutation (c.410G > A) and complained of metamorphopsia in her left eye. Examinations revealed CNV with serous retinal detachment. She was treated with as-needed injections for 2 years; however, she experienced a recurrence. Therefore, we switched to a bimonthly regimen that was continued for 6 years. In total, the patient received 34 injections of various types of anti-VEGFs over 8 years. No recurrences were noted during that time, and we have not detected any negative effects concerning the progression of visual field loss in comparison with the fellow eye.ConclusionsNo negative effects related to the progression of visual field loss were observed during continuous treatment with anti-VEGF agents for 8 years in our patient.

Ocular abnormalities in a large Western China patient cohort with Retinitis Pigmentosa

“…For several years, this information could only be attained through case reports [9,20,21]. In our study, a CNVM was observed in only one eye from one female patient (prevalence: approximately 0.09%).…”

“…Epiretinal membranes (ERMs) and cystoid macular oedema (CME) are the most common macular abnormalities in RP patients, as detected by optical coherence tomography (OCT). Other macular abnormalities also accompanied by, such as macular holes (MH), vitreomacular traction syndrome (VMT) and choroid neovascularisation membrane (CNVM) [5][6][7][8][9]. To the authors' knowledge, visual acuity and prevalence of ocular abnormalities, have not yet been reported in a large cohort of Western Chinese RP patients.…”

Ocular abnormalities in a large Western China patient cohort with Retinitis Pigmentosa

“…CNVM are rare, and until recently, no data have shown the prevalence of CNVMs in RP patients. For several years, this information could only be attained through case reports [9,14,25]. In our study, a CNVM was observed in only one eye from one female patient (prevalence: approximately 0.09%).…”

“…CNVM was de ned as cystic macular edema associated with a disruption of the Bruch membrane/retinal pigment epithelium complex, accompanied by an avascular structure emanating from the deep capillary plexus appearing as a hyper-re ective lesion connected with the subretinal pigment epithelium. And other diseases that cause macular CNV were excluded [9,14].…”

“…Epiretinal membranes (ERMs) and cystoid macular edema (CME) are the most common macular abnormalities in RP patients, as detected by optical coherence tomography (OCT). Other macular abnormalities also accompanied by, such as macular holes (MH), vitreomacular traction syndrome (VMT) and choroid neovascularisation membrane (CNVM) [5][6][7][8][9]. To the authors' knowledge, visual acuity and prevalence of ocular abnormalities, have not yet been reported in a large cohort of Western Chinese RP patients.…”