BACKGROUND: There are no guidelines on the optimal anti-vascular endothelial growth factor (anti-VEGF) monotherapy regimen for patients with polypoidal choroidal vasculopathy (PCV). In this study, we aimed to assess the comparative safety and efficacy of different treatment regimens of anti-VEGF monotherapy for PCV.
METHODS: We conducted a systematic literature search on Ovid MEDLINE, Embase, and Cochrane Library from January 2000 to May 2023 for comparative articles reporting on different treatment regimens of anti-VEGF agents in PCV. Our primary outcomes were the final best-corrected visual acuity (BCVA) and the change in BCVA from baseline. Secondary outcomes were the final retinal thickness (RT), the change in RT from baseline, the rate of polyp closure, and the incidence of adverse events.
RESULTS: A total of 10,440 studies were screened, and seven studies reporting on 636 eyes with PCV at baseline were included in this systematic review. One RCT of 53 eyes found a similar final BCVA, change in BCVA from baseline, final RT, and complete polyp closure rate between a treat-and-extend (T&E) regimen and a bimonthly fixed-dosing regimen of aflibercept. This trial also found superiority of T&E for change in RT from baseline. One observational study of 33 eyes found a similar BCVA at last study observation between a pro re nata (PRN) regimen and bimonthly fixed-dosing regimen of aflibercept. One observational study of 42 eyes found a similar change in BCVA from baseline and complete polyp closure rate between a PRN regimen and bimonthly fixed-dosing regimen of aflibercept. One RCT of 249 eyes found a similar change in BCVA and RT from baseline, as well as polyp closure, between a T&E regimen and fixed 12-week dosing regimen of conbercept. One observational study of 30 eyes found a superiority of T&E aflibercept for change in BCVA and risk of polyp recurrence, compared to a PRN regimen.
CONCLUSIONS: Overall, there is a paucity of evidence comparing various treatment regimens of anti-VEGF therapy in patients with PCV. This limited evidence suggests that current treatment regimens are similarly efficacious, though T&E aflibercept achieved superior outcomes when compared to bimonthly dosing or PRN in some individual studies. Further trials are needed to confirm or refute these findings.

There remains limited agreement regarding the efficacy and safety of different antivascular endothelial growth factor (anti-VEGF) agents for the management of polypoidal choroidal vasculopathy (PCV). Our meta-analysis compares different anti-VEGF agents for PCV treatment. Ovid MEDLINE, EMBASE, and Cochrane Library were systematically searched from January 2000 to July 2022. We included articles comparing the efficacy and safety of different anti-VEGF agents, specifically bevacizumab (BEV), ranibizumab (RAN), aflibercept AFL), and brolucizumab (BRO), for patients with PCV. 10,440 studies were identified, 122 underwent full-text review, and seven were included. One study was a randomized trial, and six were observational studies. Ranibizumab and aflibercept were associated with a similar best-corrected visual acuity (BCVA) at the last visit in three observational studies (P = 0.10), similar retinal thickness at the last visit in two observational studies (P = 0.85). One observational study comparing BEV versus RAN found comparable outcomes for final BCVA, retinal thickness, and polyp regression. One randomized trial on BRO versus AFL found comparable outcomes for improvement in BCVA, while anatomical outcomes favored BRO. The available evidence suggests that final BCVA is comparable across different anti-VEGF agents, however, further investigation is warranted due to paucity of evidence.

The purpose of this study was to provide an estimate of the number of current and future patients with polypoidal choroidal vasculopathy (PCV) in Europe. We systematically searched 11 literature databases on 18 May 2022 for studies on the prevalence of PCV among a consecutive and representative group of patients with suspected neovascular age-related macular degeneration (AMD). Prevalence of PCV in patients with suspected neovascular AMD was summarized and included in a prevalence meta-analysis. We then used current population data and population forecasts by Eurostat and the Office for National Statistics to determine current and future number of patients with neovascular AMD in Europe. Then, we calculated the number of patients with PCV with our calculated estimate of the prevalence of PCV among Europeans suspected with neovascular AMD. A total of five eligible studies were identified which included a total of 1359 patients. All these studies used the gold standard of indocyanine green angiography as a routine part of their diagnostic approach. Among patients undergoing detailed retinal examination for suspected neovascular AMD, our meta-analysis calculated the prevalence of PCV to be 8.3% (95% confidence interval: 6.8-9.8%). Our population estimates find that a total of 217,404 patients with PCV exist in Europe in the year 2022, which constitutes 0.04% of the entire population of Europe. This number is estimated to increase to 287,517 patients in the year 2040. Our estimates are important for different healthcare stakeholders, especially when planning and allocating expensive resources.

Cushing\'s syndrome is a rare disease with an endogenous cause of excess cortisol secretion. More evidence substantially links cortisol levels to the pachychoroid spectrum diseases. In this systematic review and meta-analysis, we summarize available evidence on pachychoroid spectrum diseases in patients with Cushing\'s syndrome. We performed a systematic literature search in 11 databases on 21 May 2022. Studies were considered eligible if they performed retinal examination of a consecutive group of patients with Cushing\'s syndrome using optical coherence tomography (OCT) scans. We extracted data on subfoveal choroidal thickness in patients with Cushing\'s syndrome compared to matched controls. We also extracted data on the prevalence of pachychoroid pigment epitheliopathy (PPE), central serous chorioretinopathy (CSC), and polypoidal choroidal vasculopathy (PCV). We identified six eligible studies with a total of 159 patients with Cushing\'s syndrome. On average, patients with Cushing\'s syndrome have 49.5 µm thicker subfoveal choroidal thickness compared to matched healthy individuals. Pachychoroid spectrum diseases were relatively common in these patients: PPE in 20.8%, CSC in 7.7%, and PCV in 2.8%. We conclude that there should be low threshold to recommend ophthalmic examination to patients with Cushing\'s syndrome, and that a macular OCT is recommended during this examination.

UNASSIGNED: To evaluate the diagnostic value of spectral-domain optical coherence tomography (SD-OCT) for polypoidal choroidal vasculopathy (PCV).
UNASSIGNED: A search of electronic databases was conducted from 2010 to 2021 to review the relevant literature on SD-OCT to identify PCV and other lesions causing serious or serosanguinous retinal pigment epithelial detachment (PED), specifically neovascular age-related macular degeneration (nvAMD). The QUADAS-2 scale was used to evaluate the quality of the literature. We performed a meta-analysis, including heterogeneity tests, analyze and synthesize the study data, meta-regression analysis, subgroup analysis, Fagan\'s plot, sensitivity analysis and publication bias tests.
UNASSIGNED: A total of 12 related studies involving 1,348 eyes were included in this study, and the random-effects model was used for meta-analysis. The results showed that the pooled sensitivity of SD-OCT in the diagnosis of PCV was 0.87 (95% CI: 0.84-0.89), the pooled specificity was 0.83 (95% CI: 0.80-0.86), and the pooled positive/negative likelihood ratios were 5.38 (95% CI: 3.28-8.80) and 0.16 (95% CI: 0.10-0.25), respectively. The diagnostic odds ratio (DOR) was 36.07 (95% CI: 15.98-81.40), and the area under the sROC curve (AUC) was 0.9429. When the pre-test probability was set at 20%, the post-test positive and negative probabilities were 58% and 4%, respectively. Meta-regression indicated that race was the primary source of heterogeneity (P <0.05). The Deeks\' funnel plot showed no significant publication bias in this study (P>0.05).
UNASSIGNED: SD-OCT has high sensitivity and specificity for the diagnosis of PCV, as well as significant clinical applicability. Since color fundus photography (CFP) is more clinically available and can improve the diagnostic efficacy, we recommend SD-OCT combined with CFP to diagnose PCV, especially without indocyanine green angiography (ICGA).
UNASSIGNED: https://inplasy.com/inplasy-2021-12-0048/, identifier: INPLASY2021120048.

OBJECTIVE: We conducted a systematic review to investigate the effectiveness of clinical treatments for wet age-related macular degeneration (wAMD) in Japanese patients in the decade since anti-vascular endothelial growth factor (VEGF) therapies were introduced.
METHODS: PubMed was searched for articles published in English between 1 January 2008 and 30 September 2018 using a multistring search strategy. Reviews were scanned for additional relevant studies and select gray literature was evaluated. Mean and/or median for the logarithm of the minimum angle of resolution (logMAR) visual acuity (VA), central retinal thickness (CRT), and the number of injections after 12 months of treatment were calculated using extracted data. Data were stratified by disease type and treatment modality.
RESULTS: Of 335 studies identified, 94 were selected for data extraction (147 treatment arms; typical AMD, n = 25; polypoidal choroidal vasculopathy [PCV], n = 85). Mean (median) logMAR VA was 0.44 (0.32) for typical AMD and 0.34 (0.31) for PCV; the respective mean number of anti-VEGF injections was 5.6 and 4.6. The mean CRT was approximately 220 μm for both groups. For typical AMD, anti-VEGF monotherapy resulted in better VA outcomes than photodynamic therapy (PDT) alone. For PCV, anti-VEGF monotherapy or anti-VEGF plus PDT combination therapy resulted in better VA and CRT outcomes than PDT monotherapy. Combination therapy required fewer injections than anti-VEGF monotherapy (PCV, 3.2 versus 5.3).
CONCLUSIONS: wAMD treatment has advanced dramatically in the years since anti-VEGF drugs were introduced in Japan. Discrete patient populations may benefit from differing management regimens, including the fewer injections required with combination therapy.

BACKGROUND: Polypoidal choroidal vasculopathy (PCV) is an exudative maculopathy with features similar to wet age macular degeneration. The incidence of PCV is known to be higher in the Asian population compared to Caucasians. Imaging modality is needed to make the diagnosis of PCV. Although Indocyanine green angiography (ICGA) is still the gold standard, it is not routinely performed in vitreoretinal practice. Thus another imaging modality is currently a popular research area. Spectral domain optical coherence tomography (SD-OCT) has emerged as a new imaging modality mostly available in clinics. Some studies have reported the sensitivity and specificity of SD-OCT in diagnosing PCV with different results and thresholds.
METHODS: Relevant studies from PubMed, Science Direct and Google Scholar databases were systematically searched. In random effect models using STATA 14 software, a meta-analysis was performed to determine the pooled diagnostic accuracy. QUADAS 2 was used to evaluate the risk of bias of each study by Revman 5.4 software.
RESULTS: Seven eligible studies which met the inclusion and exclusion criteria were enrolled in this study. A total of 911 eyes were included to investigate the diagnostic accuracy of SD-OCT. As a result, the pooled sensitivity was 0.91 (95% CI 0.87-0.93), specificity 0.88 (95% 0.83-0.92), positive likelihood ratio 8, negative likelihood ratio 11, the area under the summary receiver operating characteristic curve 0.95 (95% CI 0.93-0.97), and diagnostic odds ratio 71.81 (95% CI 38.89-132.74).
CONCLUSIONS: SD-OCT provided a high diagnostic value for detecting PCV. Sharply peaked pigment epithelial detachment (PED), notched PED, bubble sign, multiple PED, and double-layer sign were the most common features found in PCV.

OBJECTIVE: The aim of this article is to do a comprehensive literature review about the current understandings of the pachychoroid disease spectrum, describing its multimodal imaging analysis, pathophysiology, differential diagnosis, and current types of management.
METHODS: This comprehensive literature review was performed based on a search on the PubMed database, of relevant pachychoroid published papers according to our current knowledge.
CONCLUSIONS: The pachychoroid disease spectrum, according to some authors, includes the following: pachychoroid pigment epitheliopathy (PPE), central serous chorioretinopathy (CSC), pachychoroid neovasculopathy (PNV), polypoidal choroidal vasculopathy (PCV)/aneurysmal type 1 neovascularization (AT1), and more recently focal choroidal excavation (FCE) and peripapillary pachychoroid syndrome (PPS). Each one of these entities will be described and discussed in this article.
CONCLUSIONS: Significant advances in multimodal imaging have enabled a better understanding of the typical choroidal changes in pachychoroid disease spectrum. The clinical knowledge and managing options about this disease significantly increased in the last years. However, it is still unclear why some eyes with typical pachychoroid disease phenotype show no evidence of RPE damage and subretinal fluid (uncomplicated pachychoroid) while others present progressive tissue damage, neovascularization, and atrophy.

Polypoidal choroidal vasculopathy (PCV) is usually regarded as a subtype of choroidal neovascularization (CNV) that is secondary to age-related macular degeneration (AMD) characterized by choroidal vessel branching, ending in polypoidal lesions. Despite their close association, PCV and neovascular AMD have shown differences, especially regarding patients\' treatment response. Currently, antivascular endothelial growth factor (anti-VEGF) drugs, such as ranibizumab, bevacizumab and aflibercept, have demonstrated their efficacy in CNV patients. However, in PCV, anti-VEGF treatments have shown inconclusive results. Many genetic polymorphisms have been associated with a variable response in exudative/wet AMD patients. Thus, the aim of this study is to explore the genetic variants affecting anti-VEGF drug response in PCV patients. In this regard, we performed a systematic review and meta-analysis. We found four variants (CFH I62V, CFH Y402H, ARMS2 A69S, and HTRA1-62A/G) that have been significantly related to response. Among them, the ARMS2 A69S variant is assessed in our meta-analysis. In conclusion, in order to implement anti-VEGF pharmacogenetics in clinical routines, further studies should be performed, distinguishing physio-pathogenic circumstances between PCV and exudative AMD and the combined effect on treatment response of different genetic variants.

Aetiological processes of polypoidal choroidal vasculopathy (PCV) remains poorly understood, but several studies indicate that immunity may play a role and report elevated levels of systemic C-reactive protein (CRP). In this systematic review and meta-analysis, we summarize available evidence in the field. We searched the databases PubMed/MEDLINE, EMBASE, Web of Science and the Cochrane Central on 19 March 2020. Two independent authors reviewed the studies and extracted data. Two independent authors reviewed the studies, extracted data and evaluated risk of bias within individual studies. Studies were reviewed in the text qualitatively and measures of association were included for quantitative analyses. Results from univariate analyses and multivariate-adjusted analyses were included for separate meta-analyses to evaluate whether the association was only due to factors associated with PCV. Four studies (246 patients with PCV and 2861 control individuals) were identified and included for a qualitative and quantitative analysis. Increased CRP was associated with PCV when pooling both univariate measures (OR 3.54, 95% CI: 2.13-5.89, p < 0.0001) and multivariate-adjusted measures (OR 3.05, 95% CI: 1.56-5.98, p = 0.0011). Sensitivity analyses confirmed robustness of the results. Increased CRP is associated to PCV, even after adjusting for demographics, lifestyle factors and co-morbidities. Clinical value of CRP in relation to PCV remains unclear, but the association gives much needed insight into the aetiology of a poorly understood disease.