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Margaret Reynolds, Jose Pulido, Kevin Veverka; Ocular manifestations of systemic amyloidosis. Invest. Ophthalmol. Vis. Sci. 2016;57(12):2428.
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© ARVO (1962-2015); The Authors (2016-present)
Systemic amyloidosis describes a group of devastating conditions, which can have ophthalmic manifestations.
A retrospective chart review was undertaken including all patients with a diagnosis of light chain amyloidosis (AL), secondary amyloidosis (AA), or nonTTR familial amyloidosis who had ocular exams at Mayo Clinic between January 1, 1985 through April 1, 2014.
Eight of 68 patients, 14 eyes, with AL amyloidosis had ocular involvement secondary to conjunctival, temporal artery, extraocular muscle, trabecular meshwork, and cranial nerve deposition. One of five patients with nonTTR familial amyloidosis had gelsolin related corneal dystrophy. No patients with AA amyloidosis (n=8) had ophthalmic manifestations. Significant differences did not exist between patients with and without ophthalmic involvement for age at amyloid diagnosis, age at amyloid symptoms, survival, sex, race, age at first eye exam, visual acuity, or intraocular pressure. Comparing patients with and without ocular amyloid, patients with nonTTR familial amyloidosis (logMAR -0.01±0.09) were more likely to have a better visual acuity at first eye exam than patients with AA (0.22±0.33) or AL amyloidosis (0.20±0.38) (p<0.05).
Systemic amyloidosis can lead to ocular morbidity. AL amyloidosis patients had involvement of the temporal artery, conjunctiva, extraocular muscles, trabecular meshwork, and cranial nerves. Those with gelsolin non-TTR familial amyloidosis were susceptible to corneal dystrophy. Patients with AA amyloidosis did not manifest ophthalmic involvement. Patients with nonTTR amyloidosis had significantly better visual acuity at presentation than patients with AL or AA amyloidosis, regardless of ocular involvement of amyloid. Finally, if ocular amyloidosis is detected, patients should be referred for thorough systemic work up.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
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