• Jiangyun Luo and Yongpeng Ge.
• Department of Rheumatology and immunology, the second people's Hospital of Ningxia.
• Medicine (Baltimore). 2020 Oct 23; 99 (43): e22942.

RationaleTracheobronchial amyloidosis (TBA) associated with Sjögren syndrome is very rare. Here, we describe a case with this phenomenon, in order to better understand the condition.Patient ConcernsA 52-year-old woman presented after 6 months of coughing, sputum, and dyspnea. Chest computed tomography revealed thickened bronchial walls, which were irregular on the left side the trachea. She had a history of dry eye and dry mouth of at least 3 years' duration.DiagnosesSjögren syndrome was diagnosed based on her symptoms, ophthalmological and parotid examination, and immunological and autoantibody tests. The diagnosis of TBA was confirmed by Congo red staining of a tracheal biopsy.InterventionsThe patient was given glucocorticoids without any other immunosuppressants.OutcomesThe symptoms improved after 6 months.LessonsTBA associated with Sjögren syndrome is a rare condition. TBA is characterized by amyloid deposition to the trachea in the absence of systemic amyloidosis. Diagnosis requires tissue biopsy with demonstration of amyloid deposition.

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