Airway Amyloidosis: A Retrospective Analysis of 43 Patients

Background: Amyloidosis can involve any compartment in the thorax. We aimed to explore the clinical and radiologic presentation, treatment, and clinical course of airway amyloidosis. Methods: A computer-assisted search was performed to identify patients who had biopsy-proven airway amyloidosis and were evaluated at Mayo Clinic in Rochester, MN, from January 1, 1997 through December 31, 2019. Demographic, clinical, and radiologic features along with clinical outcomes were analyzed. Results: We identified 43 patients who had airway amyloidosis. Median age was 60 years (range: 33 to 91 y), and 58% were female. Shortness of breath (63% of patients) and cough (44%) were the most common presenting symptoms. Most patients (82%) had localized amyloidosis with light chain being the most common amyloid type; 63% had tracheobronchial amyloidosis, and 23% had tracheal and upper airway involvement. On computed tomography of the chest, the most common findings were airway wall thickening with nodularity (60% of patients), airway calcification (53%), and airway occlusion without collapse (47%). On bronchoscopy (33 patients), the extent of amyloid involvement was most commonly submucosal (n=15) or nodular (n=8). External beam radiotherapy was the most common treatment modality. Among the 30 patients who had follow-up at our institution, the prognosis appeared to depend on the extent of the disease and whether patients had localized or systemic amyloidosis. Conclusion: Computed tomography of the chest, bronchoscopy, and biopsy are needed to establish the diagnosis of airway amyloidosis, and systemic amyloidosis should be ruled out. Treatment of amyloidosis requires a multidisciplinary approach.