视网膜血管瘤样增生的临床研究进展

The progress in clinical research of retinal angiomatous proliferation

视网膜血管瘤样增生（RAP）是老年性黄斑变性（AMD）一种基因型不同的特殊亚型。主要临床表现为双眼发病、网状玻璃膜疣和视网膜色素上皮脱离。与AMD比较，视力下降更快、复发率更高。吲哚青绿血管造影是其诊断金标准。其发病机制尚不清楚，近年病理生理学研究及光相干断层扫描（OCT）血管成像加深了对该病的理解；OCT完善了对该病的分期、诊断的认识水平。RAP治疗效果与分期密切相关，抗血管内皮生长因子（VEGF）药物为一线治疗方法，光动力疗法联合抗VEGF药物能够取得较好的治疗效果。由于目前流行病学、发病机制仍不清楚，诊断及治疗方式的标准化亟待解决。

Retinal angiomatous proliferation （RAP） is a genetic distinct subgroup of exudative agerelated macular degeneration which shows a rapid and severe vision loss and high recurrence rates. The pathophysiological mechanisms of RAP is unclear. Recent histopathologic study and en face optical coherence tomography angiography have furthered our understanding of RAP. Clinical features frequently associated with RAP include bilateral disease, presence of reticular pseudodrusen and pigment epithelial detachments. Indocyanine green angiography is the gold standard diagnostic tool. Recently, more and more accurate optical coherence tomography has improved the acknowledgement of stage and diagnosis of RAP. The treatment efficacy of RAP is highly dependent on the stage. Anti-vascular endothelial growth factor therapy is currently the first line of treatment. Other treatment options including combination of photodynamic therapy with antiangiogenic agent intravitreal injections also achieve a reasonable therapeutic outcome. There remain several important questions such as pathogenesis and treatment regimen, to be answered in future RAP research studies.