Abstract
BackgroundHerein we report a case of bilateral anterior ischemic optic neuropathy (AION) showing histopathologic evidence of AL-amyloidosis of the temporal arteries. It is known that light-chain (AL) amyloidosis may rarely affect the temporal arteries, mimicking giant cell arteritis, while, to our knowledge, the association between AL-amyloidosis and AION was not previously described.Case presentationA 64 year-old woman with AL-amyloidosis secondary to a monoclonal gammopathy of undetermined significance (MGUS) referred to our hospital for acute painless drop of vision due to bilateral AION. Age greater than 50 years, high erythrocyte sedimentation rate (ESR), and bilateral AION were suggestive of giant cell arteritis (GCA). However, a temporal artery biopsy excluded GCA, showing segmental stenosis of the lumen caused by amyloidosis of the artery wall.ConclusionsThe present case shows that AL-amyloidosis may present with AION, high ESR, and temporal artery involvement, mimicking GCA. In patients with monoclonal gammopathies, C-reactive protein may be a more specific index of GCA compared with the ESR. Patient medical history and pathology are crucial for a correct diagnosis.
Highlights
We report a case of bilateral anterior ischemic optic neuropathy (AION) showing histopathologic evidence of AL-amyloidosis of the temporal arteries
The present case shows that AL-amyloidosis may present with AION, high erythrocyte sedimentation rate (ESR), and temporal artery involvement, mimicking giant cell arteritis (GCA)
In patients with monoclonal gammopathies, C-reactive protein may be a more specific index of GCA compared with the ESR
Summary
The case described presented a problematic differential between arteritic (AAION) and non-arteritic anterior optic neuropathy (NAION). Age greater than fifty years, bilateral eye involvement, and high ESR at presentation were suggestive of a diagnosis of AAION. A possible hypothesis explaining the pathogenesis of the optic nerve head ischemia is that the AL-amyloid deposition found in the temporal artery specimen may have affected the posterior ciliary arteries, causing arterial stenosis and insufficiency, or reduced adaptability to blood pressure variations. In the present case it can’t be excluded that the patient was not aware of a preexistent monolateral AION until the contralateral eye was involved. The present case shows that ALamyloidosis may present with bilateral AION, mimicking giant cell arteritis for the visual symptoms, and for high ESR values and temporal artery involvement.
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