22 Tracheobronchial Amyloidosis

Clinical Presentation

56-year-old woman with cough

Radiologic Findings

Contrast-enhanced chest CT (mediastinal window) (Figs. 22.1A, 22.1B) demonstrates abnormal soft tissue extending anterolaterally along the tracheal wall and narrowing the lumen. Note the absence of soft-tissue infiltration/obliteration of peritracheal tissue planes.

Diagnosis

Tracheobronchial Amyloidosis

Differential Diagnosis

• Primary and Secondary Neoplasia (e.g., adenoid cystic carcinoma, tracheal metastases)

• Wegener Granulomatosis

• Tracheobronchopathia Osteochondroplastica

Fig. 22.1

Discussion

Background

Amyloidosis is a rare disease that may affect the lung or tracheobronchial tree. It may occur as a primary lesion or as secondary amyloid deposition in association with chronic disease. Tracheobronchial involvement is the most common and severe form of thoracic amyloidosis. Primary tracheal amyloidosis is rare and usually involves the trachea in a slow and indolent manner.

Etiology

The etiology of tracheobronchial amyloidosis is unknown. Chronic diseases associated with secondary amyloidosis include rheumatoid arthritis, Crohn disease, ankylosing spondylitis, tuberculosis, bronchiectasis, and familial Mediterranean fever.