Abstract
Pachychoroid neovasculopathy (PNV) is a new concept of macular disorder. Some cases diagnosed as age-related macular degeneration (AMD) have been re-diagnosed as PNV. However, the biological features of PNV are still uncertain. The purpose of this study was to compare PNV and AMD by analyses focusing on von Willebrand factor (VWF) and complement factor H (CFH). Ninety-seven patients who were previously diagnosed with treatment naïve AMD were enrolled in this study. They were re-classified as either PNV or AMD based on the clinical criteria and 33 patients were classified as PNV and 64 patients as AMD. We examined the clinical data, analyzed VWF multimer and two genetic polymorphisms (I62V and Y402H) in the CFH. PNV group was significantly younger than AMD group (P = 0.001). In both I62V and Y402H, there were no significant differences between PNV and AMD while the recessive homozygous (AA) was found only in PNV group in I62V. The presence of unusually large VWF multimers (UL-VWFMs) and subretinal hemorrhages were significantly higher in PNV than in AMD (P = 0.045, P = 0.020, respectively). Thus, the residual UL-VWFMs may result in platelet thrombosis and hemorrhages in the choriocapillaris of PNV. In conclusion, our results suggest the biological differences between PNV and AMD.
Highlights
Age-related macular degeneration (AMD) is a major retinal disease in the e lderly[1]
We showed that subretinal hemorrhages were significantly higher in pachychoroid neovasculopathy’ (PNV) group than in age-related macular degeneration (AMD) group
Our results showed that subretinal hemorrhages and polypoidal choroidal vasculopathy (PCV) were significantly higher in the UL-VWFM positive group
Summary
Age-related macular degeneration (AMD) is a major retinal disease in the e lderly[1]. There are two specific types in neovascular AMD: polypoidal choroidal vasculopathy (PCV) and retinal angiomatous proliferation (RAP)[1]. Complement factor H (CFH) is a well-known risk factor for AMD5,6, and it is activated by various pathways to initiate immune responses. This activation may cause cell damage[7]. CSC occurs mostly in middle aged males and is characterized by choroidal thickening and dysfunction of the RPE which results in the presence of subretinal fluid (SRF) and RPE atrophy[19,20]. PPE is characterized by hyperplasia of the RPE and choroidal thickening without SRF, and it was suggested as an incomplete state or a pre-stage of CSC18. We consider that many cases diagnosed as AMD may be PNV and that comparisons of the biological characteristics of AMD and PNV have not been well done
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