Ocular amyloidosis, with special reference to the hereditary forms with vitreous involvement

The complex of diseases referred to as amyloidosis is characterized by the deposition of amyloid substance in various tissues. The amyloid protein differs in the various forms of amyloidosis. This variation is the basis of the differences in affected tissues and subsequent clinical dissimilarities. Vitreous involvement in amyloidosis seems to be especially linked to some of the hereditary neuropathies associated with the amyloid protein transthyretin. Characterization of the amyloid proteins during recent decades has allowed a chemical and immunologic classification of amyloid fibrils. This paper presents the basis for classification of amyloidosis, reviews the literature on ocular amyloidosis, with special reference to vitreous involvement, and summarizes clinical findings and frequency of vitreous amyloid involvement in Swedish patients with familial amyloidotic polyneuropathy.