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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Publication Details

Journal
Therapeutic Advances in Respiratory Disease
Published
2025-01-01
DOI
https://doi.org/10.1177/17534666251342145
Primary Topic
Amyloidosis: Diagnosis, Treatment, Outcomes
Type
article
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0.00
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article

Tracheobronchial amyloidosis with Sjögren’s syndrome: a case report

Derong Zhang, Chongxiang Chen, Na Zhao, C Chen et al.
Therapeutic Advances in Respiratory Disease
Amyloidosis: Diagnosis, Treatment, Outcomes
article

Tracheobronchial amyloidosis with Sjögren’s syndrome: a case report

Derong Zhang, Chongxiang Chen, Na Zhao, C Chen, Pingping Wang, Ping Peng
article en

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.

Therapeutic Advances in Respiratory DiseaseVol. 19
First Affiliated Hospital of Guangzhou Medical University (CN), State Key Laboratory of Respiratory Disease (CN), Guangzhou Medical University (CN)
Good health and well-being
Openalex Percentile: Top 90%
Amyloidosis: Diagnosis, Treatment, Outcomes
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