Tracheobronchial amyloidosis in primary Sjögren syndrome: A case report

Medicine (Baltimore). 2020 Oct 23;99(43):e22942. doi: 10.1097/MD.0000000000022942.

Abstract

Rationale: Tracheobronchial 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 concerns: A 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.

Diagnoses: Sjögren 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.

Interventions: The patient was given glucocorticoids without any other immunosuppressants.

Outcomes: The symptoms improved after 6 months.

Lessons: TBA 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.

Publication types

  • Case Reports

MeSH terms

  • Amyloidosis / diagnosis
  • Amyloidosis / etiology*
  • Biopsy
  • Bronchial Diseases / pathology*
  • Bronchoscopy / methods
  • Cough / diagnosis
  • Cough / etiology
  • Dyspnea / diagnosis
  • Dyspnea / etiology
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Middle Aged
  • Sjogren's Syndrome / complications*
  • Sjogren's Syndrome / diagnosis
  • Sjogren's Syndrome / drug therapy
  • Sputum
  • Tomography, X-Ray Computed / methods
  • Tracheal Diseases / pathology*
  • Treatment Outcome

Substances

  • Glucocorticoids