Secondary Glaucoma Accompanied with Primary Familial Amyloidosis

Abstract

Patients (22) were diagnosed as having primary familial amyloidosis by histological examination of biopsy specimens, clinical course of the disease and family history, having a focus in the Nagano Prefecture in Japan. All patients had the Andrade type of neuropathy. Of 22 patients, 15 underwent ophthalmological examination, and secondary glaucoma, due to primary familial amyloidosis, was found in 4 patients. The clinical features of the secondary glaucoma were: the systemic symptoms of primary familial amyloidosis; being relatively older and having the disease longer; vitreous opacities; wide open angle with pigment deposition in gonioscopy; white flocculent materials in the pupillary margin; and flaky substances on the surface of the lens. The last three ophthalmological symptoms were very similar to those of glaucoma capsulare. The tissue specimens obtained at the time of antiglaucoma surgery were subjected to light microscopy and EM. Amyloid fibrils were found in the iris and trabeculum, and the pathogenesis of secondary glaucoma may be due to deposition of amyloid fibrils in the trabecular meshwork.