{"title":"气管支气管淀粉样变合并Sjögren综合征1例。","authors":"Chongxiang Chen, Pingping Wang, Na Zhao, Derong Zhang, Cuifen Chen, Ping Peng","doi":"10.1177/17534666251342145","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":22884,"journal":{"name":"Therapeutic Advances in Respiratory Disease","volume":"19 ","pages":"17534666251342145"},"PeriodicalIF":3.0000,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12102560/pdf/","citationCount":"0","resultStr":"{\"title\":\"Tracheobronchial amyloidosis with Sjögren's syndrome: a case report.\",\"authors\":\"Chongxiang Chen, Pingping Wang, Na Zhao, Derong Zhang, Cuifen Chen, Ping Peng\",\"doi\":\"10.1177/17534666251342145\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":22884,\"journal\":{\"name\":\"Therapeutic Advances in Respiratory Disease\",\"volume\":\"19 \",\"pages\":\"17534666251342145\"},\"PeriodicalIF\":3.0000,\"publicationDate\":\"2025-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12102560/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Therapeutic Advances in Respiratory Disease\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1177/17534666251342145\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/5/23 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q2\",\"JCRName\":\"RESPIRATORY SYSTEM\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Therapeutic Advances in Respiratory Disease","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1177/17534666251342145","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/5/23 0:00:00","PubModel":"Epub","JCR":"Q2","JCRName":"RESPIRATORY SYSTEM","Score":null,"Total":0}
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.
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.