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由轻链淀粉样变性引起的血管周和肺泡间隔沉积导致的气肿性肺病变。

Emphysematous Lung Lesions Caused by Perivascular and Alveolar-Septal Deposition of Amyloid Light-Chain Amyloidosis.

机构信息

Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mayo Clinic, Rochester, MN.

Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mayo Clinic, Rochester, MN.

出版信息

Chest. 2021 Aug;160(2):e169-e171. doi: 10.1016/j.chest.2021.03.038.

Abstract

Pulmonary amyloidosis, whether isolated or seen as part of systemic amyloidosis, has a variety of radiographic manifestations. Known parenchymal lung findings include reticulonodular opacities, diffuse interstitial infiltrates, or cystic lesions. Here, we present a case of systemic amyloid light-chain (AL) amyloidosis presenting with severe exertional dyspnea and emphysematous lung lesions on chest CT, a finding described only once before. Although factors that influence the pattern of pulmonary amyloid deposition remain unclear, CT image findings typically reflect the histopathologic patterns of deposition. In this case, we hypothesize that the emphysematous changes in the lower lung zones are likely a manifestation of severe alveolar-septal involvement. This case suggests that radiographic findings of pulmonary amyloidosis are not limited to the more common findings of reticular opacities or interstitial infiltrates. Emphysematous changes are possible, and clinicians should maintain a broad differential when seen in the setting of dyspnea.

摘要

肺淀粉样变性,无论是孤立性的还是作为系统性淀粉样变性的一部分,都有多种放射影像学表现。已知的肺实质病变包括网状结节状混浊、弥漫性间质浸润或囊性病变。在这里,我们报告了一例系统性轻链(AL)淀粉样变性的病例,该病例表现为严重的劳力性呼吸困难和胸部 CT 上肺气肿样肺病变,这种表现以前只描述过一次。尽管影响肺淀粉样物质沉积模式的因素尚不清楚,但 CT 图像表现通常反映了沉积的组织病理学模式。在这种情况下,我们假设下肺区的肺气肿样改变可能是严重肺泡-间隔受累的表现。该病例表明,肺淀粉样变性的放射影像学表现不仅限于更常见的网状混浊或间质浸润表现。肺气肿样改变是可能的,当在呼吸困难的情况下看到时,临床医生应该保持广泛的鉴别诊断。

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