Schweitzer C
Service d'ophtalmologie, CHU de Bordeaux, 33000 Bordeaux, France; UMR 1219, Inserm, Bordeaux Population Health Research Center, team LEHA, université Bordeaux, 33000 Bordeaux, France.
J Fr Ophtalmol. 2018 Jan;41(1):78-90. doi: 10.1016/j.jfo.2017.09.003. Epub 2018 Jan 10.
Pseudoexfoliation syndrome is an age-related systemic disease that mainly affects the anterior structures of the eye. Despite a worldwide distribution, reported incidence and prevalence of this syndrome vary widely between ethnicities and geographical areas. The exfoliative material is composed mainly of abnormal cross-linked fibrils that accumulate progressively in some organs such as the heart, blood vessels, lungs or meninges, and particularly in the anterior structures of the eye. The exact pathophysiological process still remains unclear but the association of genetic and environmental factors are thought to play a role in the development and progressive extracellular accumulation of exfoliative material. Hence, LOXL1 gene polymorphisms, responsible for metabolism of some components of elastic fibers and extracellular matrix, and increased natural exposure to ambient ultraviolet or caffeine consumption have been associated with pseudoexfoliation syndrome. Ophthalmological manifestations are commonly bilateral with an asymmetric presentation and can lead to severe visual impairment and blindness more frequently than in the general population, mainly related to glaucoma and cataract. Pseudoexfoliation glaucoma is a major complication of pseudoexfoliation syndrome and represents the main cause of identifiable glaucoma worldwide. Visual field progression is more rapid than that observed in primary open angle glaucoma, and filtering surgery is more frequently required. Nuclear cataract is more frequent and occurs earlier than in the general population. Owing to poorer pupil dilation and increased zonular instability, cataract surgery with pseudoexfoliation is associated with a 5- to 10-fold increase in surgical complications compared to cataract surgery without pseudoexfoliation. Some specific treatments targeting production, formation or accumulation of exfoliative material could improve the prognosis of this syndrome.
假性剥脱综合征是一种与年龄相关的全身性疾病，主要影响眼部前部结构。尽管该综合征在全球范围内均有分布，但不同种族和地理区域报告的发病率和患病率差异很大。剥脱物质主要由异常交联的纤维组成，这些纤维在心脏、血管、肺或脑膜等一些器官中逐渐积累，尤其是在眼部前部结构中。确切的病理生理过程仍不清楚，但遗传和环境因素的关联被认为在剥脱物质的形成和细胞外渐进性积累中起作用。因此，负责弹性纤维和细胞外基质某些成分代谢的赖氨酰氧化酶1（LOXL1）基因多态性，以及自然环境中紫外线暴露增加或咖啡因摄入，都与假性剥脱综合征有关。眼科表现通常为双侧性且不对称，与普通人群相比，更易导致严重视力损害和失明，主要与青光眼和白内障有关。假性剥脱性青光眼是假性剥脱综合征的主要并发症，是全球可识别青光眼的主要原因。其视野进展比原发性开角型青光眼更快，更常需要进行滤过手术。核性白内障比普通人群更常见且发病更早。由于瞳孔散大较差和悬韧带稳定性增加，与无假性剥脱的白内障手术相比，伴有假性剥脱的白内障手术并发症增加5至10倍。一些针对剥脱物质产生、形成或积累的特定治疗方法可能会改善该综合征的预后。
