Corneal endothelial cell‐matrix interactions in Fuchs corneal endothelial dystrophy

Abstract

AbstractFuchs endothelial corneal dystrophy (FECD) is characterized by progressive loss of corneal endothelial cells (CECs) and excessive deposition of extracellular matrix, resulting in Descemet's membrane thickening and formation of excrescences (guttae) on its posterior surface. The role of the endothelium being to keep the cornea dehydrated and transparent, loss of corneal endothelial function leads to corneal edema and vision loss. FECD etiology is multifactorial, involving a combination of parameters such as age, environmental factors and genetic predisposition, and genetic and proteomic studies have given rise to several hypotheses regarding the pathogenesis of FECD. Up to recently, however, most of the work has focused on CECs as the primary site of the disease, while several clues suggest that interactions between CECs and their environment are significantly involved in the development of FECD. The influence of the altered topography and composition of diseased Descemet's membrane and guttae on CECs in FECD will be discussed.