Tracheobronchial Amyloidosis: Clinical Experience From 2018 to 2023 at a National Respiratory Centre

Tracheobronchial amyloidosis is a rare disease. This study aimed to assess the clinical presentation, radiological findings, bronchoscopic features, pathological findings and management approach at a national respiratory centre in China.

We retrospectively analysed biopsy-confirmed tracheobronchial amyloidosis cases from 2018 to 2023 using electronic medical records. Data on demographics, clinical manifestations, investigational findings, management and prognosis were analysed.

Six patients (2 women and 4 men, mean age 55.5 years) were diagnosed with tracheobronchial amyloidosis. Common symptoms included cough, sputum and dyspnoea. None of the patients had evidence of extra-pulmonary amyloidosis. Diagnosis was confirmed via tracheobronchial biopsy. Precursor proteins included AL-λ and AL-κ in 2 patients, AL-κ and ATTR in one patient. The median delay from symptoms onset to diagnosis was 18 months while computerised tomography (CT) imaging expediated diagnosis of tracheobronchial amyloidosis, with a median time from CT thorax to diagnostic bronchoscopy of 2 days in four cases, with two cases lacking detailed information on the date of the initial CT. Treatments included bronchoscopic intervention in three patients and observation in three patients.

Patients with tracheobronchial amyloidosis often present with symptoms resembling those of other airway disorders. Considering this condition in differential diagnosis is crucial when standard treatments fail. Chest CT facilitates prompt referral for diagnostic bronchoscopy. No curative therapy is currently available.