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结节性肺淀粉样变性与干燥综合征相关。

Nodular Pulmonary Amyloidosis Associated with Sjögren's Syndrome.

机构信息

Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Japan.

Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Japan.

出版信息

Intern Med. 2022 Mar 15;61(6):877-881. doi: 10.2169/internalmedicine.8169-21. Epub 2021 Aug 31.

Abstract

Amyloidosis is a rare disease characterized by the deposition of abnormal proteins in extracellular tissues. We herein report a case with instructive radiologic features of nodular pulmonary amyloidosis associated with Sjögren's syndrome. A 67-year-old woman was referred to our department because of an abnormal chest radiograph. Chest computed tomography revealed multiple round cysts accompanied by calcified nodules. The patient was clinically diagnosed with primary Sjögren's syndrome and pathologically diagnosed with nodular pulmonary amyloidosis (light chain, kappa). Although multiple lung cysts have many etiologies, the presence of calcified nodules associated with multiple lung cysts is useful for narrowing down the differential diagnosis.

摘要

淀粉样变性是一种罕见的疾病,其特征是异常蛋白质在细胞外组织中的沉积。本文报告一例伴有干燥综合征的结节性肺淀粉样变性的具有指导意义的影像学特征。一名 67 岁女性因胸片异常就诊于我院。胸部 CT 显示多个圆形囊肿伴有钙化结节。患者临床诊断为原发性干燥综合征,病理诊断为结节性肺淀粉样变性(轻链,κ)。尽管多发性肺囊肿有多种病因,但伴有多发性肺囊肿的钙化结节的存在有助于缩小鉴别诊断范围。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/abe4/8987246/7c91231a9760/1349-7235-61-0877-g001.jpg

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