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AL 淀粉样变性累及颞动脉:巨细胞动脉炎的重要鉴别诊断。病例报告及文献复习。

Temporal artery involvement in AL amyloidosis: an important differential diagnosis for giant cell arteritis. A case report and literature review.

机构信息

Department of Rheumatology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan.

Department of Neurology and Rheumatology, Shinshu University School of Medicine, Matsumoto, Japan.

出版信息

Mod Rheumatol Case Rep. 2020 Jan;4(1):90-94. doi: 10.1080/24725625.2019.1650993. Epub 2019 Aug 13.

Abstract

AL amyloidosis (AL) is a systemic disorder due to extracellular tissue deposition of amyloid fibrils, composed of immunoglobulin light chains. Since the description of AL involving temporal arteries in 1986, this disorder has been known as one of the differential diagnoses of giant cell arteritis (GCA). We encountered a case of an elderly female presenting with headache and tender and enlarged temporal arteries, that was pathologically diagnosed with temporal artery involvement of AL due to Bence-Jones-type MM. To our knowledge, this was the first case of AL with temporal artery involvement in Japan, that presented with GCA-like features. Literature review of AL cases with temporal artery involvement showed close similarity between these disorders, but suggested that vasculature involvement (extremity claudication, kidney or heart), macroglossia, carpal tunnel syndrome and normal or low (<0.5 mg/dL) CRP levels may predict AL rather than GCA. Physicians should keep in mind that AL involving temporal arteries can be a pitfall in the diagnosis of GCA, as seen in our and previous cases.

摘要

AL 淀粉样变性(AL)是一种由于免疫球蛋白轻链组成的细胞外组织沉积的淀粉样纤维而导致的系统性疾病。自 1986 年描述了涉及颞动脉的 AL 以来,这种疾病已被认为是巨细胞动脉炎(GCA)的鉴别诊断之一。我们遇到了一位老年女性,表现为头痛和触痛及颞动脉肿大,经病理诊断为由于 Bence-Jones 型 MM 导致的颞动脉受累的 AL。据我们所知,这是首例在日本出现 GCA 样特征的伴有颞动脉受累的 AL 病例。对伴有颞动脉受累的 AL 病例的文献回顾表明这些疾病之间存在密切的相似性,但提示血管受累(四肢跛行、肾脏或心脏)、巨舌、腕管综合征和正常或低(<0.5mg/dL)CRP 水平可能预示着 AL 而非 GCA。医生应牢记,如我们和以前的病例所示,累及颞动脉的 AL 可能是 GCA 诊断中的一个陷阱。

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