CTICasesPrimary Tracheobronchial Amyloidosis
Imaging findings
CT shows diffuse, circumferential soft tissue thickening and narrowing of the trachea and main bronchi without calcification, in a patient presenting with stridor. Bronchoscopic biopsy was congo red positive, demonstrating apple-green birefringence.
Key takeaways
Primary tracheobronchial amyloidosis is an isolated deposition of amyloid in the central airways. It typically presents with circumferential narrowing of the trachea and main bronchi. It carries a high long-term morbidity and mortality due to progressive airway obstruction.
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