Ophthalmology, University Hospital of Parma, Via Gramsci 14, Parma, PR 43100, Italy.
BMC Ophthalmol. 2013 Dec 20;13:82. doi: 10.1186/1471-2415-13-82.
Herein 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.
A 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.
The 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.
在此，我们报告一例双侧前部缺血性视神经病变（AION），其颞动脉存在 AL 淀粉样变性的组织病理学证据。已知轻链（AL）淀粉样变性可能很少累及颞动脉，类似于巨细胞动脉炎，然而，据我们所知，AL 淀粉样变性与 AION 之间的关联以前并未描述过。
一位 64 岁女性因意义未明的单克隆丙种球蛋白病（MGUS）继发 AL 淀粉样变性，因双侧 AION 导致急性无痛性视力下降而就诊于我院。年龄大于 50 岁、红细胞沉降率（ESR）高和双侧 AION 提示巨细胞动脉炎（GCA）。然而，颞动脉活检排除了 GCA，显示动脉壁淀粉样变性导致管腔节段性狭窄。
本病例表明，AL 淀粉样变性可能表现为 AION、ESR 高和颞动脉受累，类似于 GCA。在单克隆丙种球蛋白病患者中，与 ESR 相比，C 反应蛋白可能是 GCA 的更特异指标。患者的病史和病理学对正确诊断至关重要。
