Ranibizumab for choroidal neovascularization secondary to pseudoxanthoma elasticum: 4-year results from the PIXEL study in France

Abstract

PurposeTo evaluate the long-term effectiveness and safety of ranibizumab 0.5 mg in patients with choroidal neovascularization (CNV) secondary to pseudoxanthoma elasticum (PXE) in a real-world setting.MethodsA descriptive, observational, multicenter study in a retrospective and prospective cohort was conducted in France that included patients who had received at least one injection of ranibizumab 0.5 mg during the period October 2011 to October 2014, for CNV secondary to PXE. Eligible patients were identified by review of medical records or during routine consultations. The main objectives were to describe patient characteristics, assess changes in best-corrected visual acuity [VA, Early Treatment Diabetic Retinopathy Study (ETDRS) letters] over time, the number and reasons for ranibizumab treatment and overall safety.ResultsOf the 72 enrolled patients (98 eyes) from 23 centers, 39 (54.2%) were male and mean [±standard deviation (SD)] age was 59.6 (±8.3) years. The mean VA was 64.6 letters at the first ranibizumab injection, which was maintained at the 1-year follow-up (64.7 letters). Thereafter, the mean VA was stable until the 4-year follow-up. At 4 years, the proportion of eyes with VA gain of ≥15 letters was 3/19 (15.8%) and stable VA (change between −15 and +15 letters) was 10/19 (52.6%). Mean (±SD) annual number of ranibizumab injections was 4.1 (±4.0), lower in the second versus first year. The most common reason for ranibizumab treatment was progression of neovascular activity (42.9%). No deaths or new safety findings were reported.ConclusionsIn patients with CNV secondary to PXE, ranibizumab 0.5 mg resulted in stable VA over 4 years with a limited number of injections. Safety findings were consistent with the established safety profile of ranibizumab.

Long-Term Effect of Anti-Vascular Endothelial Growth Factor (Anti-VEGF) Injections in Choroidal Neovascularization Secondary to Angioid Streaks

“…In this study, patients had a mean age of 53.2 ± 12.1 years at the time of CNV diagnosis/treatment initiation, which is consistent with previous studies [ 12 , 16 , 17 ]. Moreover, the mean age of patients with PXE and angioid streaks complicated with CNV seems to be significantly lower than in patients with no PXE, 49.0 ± 10.5 years vs. 58.1 ± 12.4, P =0.023.…”

“…The PIXEL study included 98 eyes of 72 patients with PXE and CNV secondary to angioid streaks treated with ranibizumab [ 12 ]. The mean BCVA was 64.6 letters at baseline and 64.7 letters at 12 months, and it stabilized until the 4-year follow-up [ 12 ].…”

Progression from Type 2 Macular Neovascularization to Fibrovascular Pigment Epithelial Detachment

“…From these results, we hypothesize that progression from type 2 MNV to fibrovascular PED is associated with a good visual prognosis, at least on short follow up basis. Results from a recent study confirmed that patients with MNV secondary to angioid streaks showed a regression or a stabilization of the lesions with a limited number of intravitreal anti-VEGF injections [26].…”

Clinical characteristics and treatment outcomes of angioid streak associated choroidal neovascular membrane (AS-CNV): a Zambian case series

“…This conforms to the revised criteria for diagnosing PXE where comet lesions constitute only minor eye criteria [ 10 ]. Bilateral CNV development was seen in one patient at an interval of 12 months; the available literature predicts risk of bilateralization of CNV within a period of 18-24 months in AS [ 11 ]. Anti-VEGF therapy is the treatment of choice as it can stabilize and even improve vision in CNV associated with angioid streaks [ 12 ].…”