Ocular lipid deposition and hyperlipoproteinaemia

Abstract

In all species there are potential ocular manifestations when circulating lipoproteins are raised and these may be transient or permanent Many factors, both systemic and local, influence lipid influx and accumulation (progression) and lipid mobilisation and efflux (regression). In both humans and animals some types of lipid deposition will regress if the local and systemic factors involved in pathogenesis can be modified. There are inescapable parallels with the same phenomena in other tissues.Three types of corneal lipid deposition have been linked with hyperlipoproteinaemia. In corneal arcus, lipid is deposited preferentially in the warmest part of the cornea initially and, in people, the lipid remains almost exclusively extracellular. In animals, corneal arcus is associated with initial extracellular lipid deposition followed by the appearance of intracellular lipid and vascularisation, so that established corneal arcus tends to become more typical of lipid keratopathy. In humans, hyperlipoproteinaemia may be an associated systemic factor and early onset corneal arcus is a recognised feature of certain primary hyperlipoproteinaemias and their secondary phenotypes. In dogs, corneal arcus is always associated with hyperlipoproteinaemia. Corneal vascularisation is a ubiquitous feature of lipid keratopathy in all species and both necrotic fibroblasts and foam cells are common in progressive lesions. The extent and position of lipid deposition and the evolution of lipid keratopathy can be related to local ocular disease and circulating lipids and lipoproteins. Many aspects of the pathogenesis of lipid keratopathy are similar to those of atherogenesis. Hyperlipoproteinaemia, especially hypercholesterolaemia is the commonest systemic abnormality. In crystalline stromal dystrophy (Schnyder's crystalline stromal dystrophy) of the cornea there is no inflammatory element and no vascularisation. The dystrophy is associated with accumulation of lipid within the corneal fibroblasts, but typical foam cells are absent, the crystalline opacity involves the coolest part of the cornea, correlates with local fibroblast death, and is always bilateral. Hyperlipoproteinaemia, may be present, but this is not universally so.The objective of this paper is to evaluate the factors that may influence ocular involvement in hyperlipoproteinaemia. A comparative approach, utilising information available from studies of both ocular and non-ocular tissues, aids elucidation of the complex pathogenesis.