OCULAR MANIFESTATIONS OF SYSTEMIC AMYLOIDOSIS. Issue 7 (July 2018)

• Record Type: Journal Article
• Authors: Reynolds, Margaret M.; Veverka, Kevin K.; Gertz, Morie A.; Dispenzieri, Angela; Zeldenrust, Steven R.; Leung, Nelson; Pulido, Jose S.
• Is Part Of: Retina. Volume 38:Issue 7(2018)
• Publication Date: 2018-07
• Subjects: ApoA1 -- ApoA2 -- beta-2 microglobulin -- gelsolin -- light-chain (AL) amyloidosis -- lysozyme -- nontransthyretin familial amyloidosis -- secondary (AA) amyloidosis -- serum amyloid A (SAA) -- systemic amyloidosis; Retina -- Diseases -- Periodicals; Retinal Diseases; Vitreous Body; 617.735
• DOI: 10.1097/IAE.0000000000001901
• Languages: English
• ISSNs: 0275-004X

Abstract

Abstract : Purpose: To describe ophthalmic manifestations of systemic amyloidosis, a group of devastating conditions. Methods: A retrospective chart review including patients who had ocular examinations at Mayo Clinic between January 1, 1985, and April 1, 2014, and a diagnosis of light-chain (AL), secondary (AA), or nontransthyretin familial amyloidosis was undertaken. Sixty-eight patients with AL amyloidosis, eight patients with AA amyloidosis, and five patients with nontransthyretin familial amyloidosis were included. Results: Of 68 patients, 8 patients (14 eyes) with AL amyloidosis had ocular involvement secondary to conjunctiva, temporal artery, extraocular muscle, trabecular meshwork, and cranial nerve deposition. One of the five patients with nontransthyretin familial amyloidosis had gelsolin-related corneal dystrophy. No patients with AA amyloidosis (n = 8) had ophthalmic manifestations. Conclusion: Systemic amyloidosis can lead to ocular morbidity. Patients with AL amyloidosis had involvement of the temporal artery, conjunctiva, extraocular muscles, trabecular meshwork, and cranial nerves. Those with gelsolin nontransthyretin familial amyloidosis were susceptible to corneal dystrophy. Patients with AA amyloidosis did not manifest ophthalmic involvement. Finally, if ocular amyloidosis is detected, patients should be referred for systemic workup. Abstract : Systemic amyloidosis can have associated ocular involvement. Light-chain amyloidosis can lead to involvement of the temporal artery, conjunctiva, extraocular muscles, trabecular meshwork, and cranial nerves. Gelsolin familial amyloidosis can lead to corneal dystrophy. Secondary amyloidosis does not typically cause ocular manifestations.