Amyloidosis and Ocular Involvement: an Overview.

摘要: Purpose: To describe the ophthalmic manifestations of amyloidosis and corresponding therapeutic measures.Methods: The 178 patients included in study had different types amyloidosis, diagnosed at a single internal medicine institution (Bari, Italy). provide comprehensive review that can be associated with ocular involvement, images clinical descriptions structurally related to gelsolin, keratoepithelin lactoferrin were obtained collaborations ophthalmology departments hospitals Mainz (Germany) Helsinki (Finland).Results: Overall, morbidity was detected 41 (23%). AL 18 systemic disease, 3 multiple myeloma, 11 localized amyloidosis. AA 2 rheumatoid arthritis Behcet syndrome, transthyretin 4 patients. treatment is based on chemotherapy suppress production amyloidogenic L-chains surgical excision orbital or conjunctival masses. managed by targeting underlying condition. Vitreous opacities additional findings involvement indicate need for pars plana vitrectomy. Gelsolin characterized lattice corneal polyneuropathy, results recurrent keratitis scarring, such keratoplasty inevitable. In dystrophies amyloid deposits fragments, transparency compromised congophilic material subepithelial layer deep stroma. Patients established are treated transplantation, but prognosis poor because possible after surgery. gelatinous drop-like dystrophy, fibrils accumulate beneath epithelium consist severely impair visual acuity. Keratoplasty its variants performed rehabilitation.Conclusion: A routine follow-up recommended all suspected independent biochemical type amyloid. Close collaboration between ophthalmologist internist will facilitate more precise diagnosis allow multidisciplinary management these patients.Abbreviations: CD: dystrophy; CLA: amyloidosis; CNS: central nervous system; CT: computed tomography; FAP: familial amyloidotic polyneuropathy; GDLCD: GLN: gelsolin; LCD: MRI: magnetic resonance imaging; OLT: orthotopic liver transplantation; TEM: transmission electron microscopy; TGFBI: transforming growth factor β induced; TTR: transthyretin.