title: Secondary glaucoma accompanied with primary familial amyloidosis.
abstract: 22 patients were diagnosed as having primary familial amyloidosis by the histological examination of biopsy specimens, clinical course of the disease, and family history, having a focus in the Nagano Prefecture in Japan. All patients belonged to the Andrade type of the neuropathy form in the primary familial amyloidosis. 15 out of 22 patients underwent ophthalmological examination, and secondary glaucoma, due to primary familial amyloidosis, was found in 4 patients. The clinical characteristic features of the secondary glaucoma were described as follows: (1) having the systemic symptoms of primary familial amyloidosis; (2) belonging to relatively older age groups and having a longer duration of the course of the disease; (3) having vitreous opacities; (4) having the wide open angle with pigment deposition in the gonioscopical examination; (5) having white flocculent materials in the pupillary margin, and (6) having flaky substances on the surface of the lens. The ophthalmological symptoms mentioned in 4, 5, and 6 were very similar to those of glaucoma capsulare. The tissue specimens obtained at the time of antiglaucoma surgery were subjected to light and electron microscopy. Amyloid fibrils were found in the iris and trabeculum, and the pathogenesis of secondary glaucoma was speculated to be a deposition of amyloid fibrils in the trabecular meshwork.
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000150:complications / Q000235:genetics* / Q000473:pathology; D005123:Eye / Q000648:ultrastructure; D005260:Female; D005901:Glaucoma / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree