Retinal Pigment Epithelial Dystrophy (RPED)

Retinal pigment epithelial dystrophy (RPED) describes a disease of the retinal pigment epithelium, marked by the pathological accumulation of lipofuscin and associated with more widespread secondary retinal degeneration. This condition has also been referred to as “central progressive retinal atrophy” (CPRA). Clinically, this disease manifests as decreased visual function (which may progress to outright blindness), subtle neurological defects (notably proprioceptive deficits) and/or secondary cataract formation. Fundoscopically, disease presentation is characterized by the (bilateral) presence of scattered tan to brown foci, which may coalesce with time. More generalized secondary retinal degeneration typically ensues. Similarities between this disease and deficiencies in the availability and absorption/metabolism of vitamin E have also been noted. Commonly affected breeds include the Labrador Retriever, Golden Retriever, Cocker Spaniel, Briard and the collie breeds, notably in European populations.