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 tracheo-bronchial
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.
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© 2000 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.