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合并干燥综合征的气管支气管淀粉样变:一例报告

Tracheobronchial amyloidosis with Sjögren's syndrome: a case report.

作者信息

Chen Chongxiang, Wang Pingping, Zhao Na, Zhang Derong, Chen Cuifen, Peng Ping

机构信息

Guangzhou Development District Hospital, Guangzhou Huangpu District People's Hospital, Guangzhou, Guangdong Province, China.

State Key Laboratory of Respiratory Disease, National Clinical Research Center for Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong Province, China.

出版信息

Ther Adv Respir Dis. 2025 Jan-Dec;19:17534666251342145. doi: 10.1177/17534666251342145. Epub 2025 May 23.

Abstract

We report a case of tracheobronchial amyloidosis (TBA) in a 55-year-old woman with newly diagnosed primary Sjögren's syndrome (SS), presenting with persistent cough, hemoptysis, and dry mucosal symptoms. Chest CT showed thickened airway walls and cystic lung changes, while bronchoscopy revealed nodular lesions with exposed vessels. Congo red staining confirmed amyloid deposition with κ light-chain dominance, consistent with AL amyloidosis. Despite immunosuppressive therapy, airway lesions persisted, highlighting the challenge of managing localized amyloidosis in SS. This case underscores the need for early recognition of TBA in SS patients presenting with respiratory symptoms.

摘要

我们报告了一例55岁新诊断为原发性干燥综合征(SS)的女性气管支气管淀粉样变(TBA)病例,该患者表现为持续咳嗽、咯血和黏膜干燥症状。胸部CT显示气道壁增厚和肺部囊性改变,而支气管镜检查发现有血管外露的结节性病变。刚果红染色证实淀粉样蛋白沉积以κ轻链为主,符合AL淀粉样变。尽管进行了免疫抑制治疗,气道病变仍持续存在,凸显了在SS中管理局限性淀粉样变的挑战。该病例强调了对出现呼吸道症状的SS患者早期识别TBA的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c474/12102560/e4f886c2f734/10.1177_17534666251342145-fig1.jpg

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