Department of Ophthalmology and Visual Sciences, University of British Columbia (UBC), Vancouver, BC, Canada.
International Collaboration on Repair Discoveries (ICORD), Vancouver Coastal Health Research Institute, University of British Columbia, Vancouver, BC, Canada.
Front Immunol. 2024 Jul 18;15:1421175. doi: 10.3389/fimmu.2024.1421175. eCollection 2024.
Age-related macular degeneration (AMD), a prevalent and progressive degenerative disease of the macula, is the leading cause of blindness in elderly individuals in developed countries. The advanced stages include neovascular AMD (nAMD), characterized by choroidal neovascularization (CNV), leading to subretinal fibrosis and permanent vision loss. Despite the efficacy of anti-vascular endothelial growth factor (VEGF) therapy in stabilizing or improving vision in nAMD, the development of subretinal fibrosis following CNV remains a significant concern. In this review, we explore multifaceted aspects of subretinal fibrosis in nAMD, focusing on its clinical manifestations, risk factors, and underlying pathophysiology. We also outline the potential sources of myofibroblast precursors and inflammatory mechanisms underlying their recruitment and transdifferentiation. Special attention is given to the potential role of mast cells in CNV and subretinal fibrosis, with a focus on putative mast cell mediators, tryptase and granzyme B. We summarize our findings on the role of GzmB in CNV and speculate how GzmB may be involved in the pathological transition from CNV to subretinal fibrosis in nAMD. Finally, we discuss the advantages and drawbacks of animal models of subretinal fibrosis and pinpoint potential therapeutic targets for subretinal fibrosis.
年龄相关性黄斑变性(AMD)是一种常见的进行性黄斑部退行性疾病,是发达国家老年人致盲的主要原因。晚期包括新生血管性 AMD(nAMD),其特征为脉络膜新生血管(CNV),导致视网膜下纤维化和永久性视力丧失。尽管抗血管内皮生长因子(VEGF)治疗在稳定或改善 nAMD 视力方面有效,但 CNV 后发生的视网膜下纤维化仍然是一个重大问题。在这篇综述中,我们探讨了 nAMD 中视网膜下纤维化的多方面问题,重点关注其临床表现、危险因素和潜在的病理生理学。我们还概述了肌成纤维细胞前体的潜在来源和招募及转分化的炎症机制。特别关注肥大细胞在 CNV 和视网膜下纤维化中的潜在作用,重点是肥大细胞介质,类胰蛋白酶和颗粒酶 B。我们总结了我们关于 GzmB 在 CNV 中的作用的发现,并推测 GzmB 如何参与 nAMD 中从 CNV 到视网膜下纤维化的病理转化。最后,我们讨论了视网膜下纤维化的动物模型的优缺点,并指出了潜在的治疗靶点。