Objective: To evaluate the presentation and clinical course of patients with tracheobronchial amyloidosis.
Patients and methods: We retrospectively reviewed the records of all patients with biopsy-proven tracheobronchial amyloidosis who were evaluated at the Mayo Clinic in Rochester, Minn, between 1973 and 1999. All relevant information, such as clinical, historical, demographic, laboratory, and histological data, was examined.
Results: Tracheobronchial amyloidosis was diagnosed in 17 patients (9 women and 8 men), with a mean age of 56.6 years. The most common symptoms at presentation were dyspnea, cough, hemoptysis, and hoarseness. None of the 14 patients who underwent investigation for systemic amyloidosis had any evidence of disease outside the tracheobronchial system except for a man in whom multiple myeloma had been diagnosed 3 years before the development of respiratory disease. Treatment of tracheobronchial amyloidosis consisted of laser or forceps resection, external radiation, systemic therapy, or observation. Two patients died of complications directly related to their disease.
Conclusions: Patients presenting with tracheobronchial amyloidosis have symptoms similar to those caused by various airway disorders. Tracheobronchial amyloidosis is not typically associated with systemic amyloidosis or pulmonary parenchymal involvement. It often recurs and despite repeated attempts with bronchoscopic techniques, airway compromise remains a major problem.