气管支气管淀粉样变合并Sjögren综合征1例。

IF 3 3区 医学 Q2 RESPIRATORY SYSTEM
Chongxiang Chen, Pingping Wang, Na Zhao, Derong Zhang, Cuifen Chen, Ping Peng
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引用次数: 0

摘要

我们报告一例气管支气管淀粉样变(TBA)在一个55岁的女性新诊断原发性Sjögren综合征(SS),表现为持续咳嗽,咯血,和干粘膜症状。胸部CT显示气道壁增厚及肺囊性改变,支气管镜显示结节状病变及血管外露。刚果红染色证实淀粉样蛋白沉积以κ轻链为主,与AL淀粉样变性一致。尽管免疫抑制治疗,气道病变持续存在,突出了控制SS中局限性淀粉样变性的挑战。该病例强调了早期识别出现呼吸道症状的SS患者的TBA的必要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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

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

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

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

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.

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来源期刊
CiteScore
6.90
自引率
0.00%
发文量
57
审稿时长
15 weeks
期刊介绍: Therapeutic Advances in Respiratory Disease delivers the highest quality peer-reviewed articles, reviews, and scholarly comment on pioneering efforts and innovative studies across all areas of respiratory disease.
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