대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Pulmonary Amyloidosis

Radiologic Findings

The nonenhanced axial CT scan shows circumferential thickening and calcifications of the both main bronchi, upper, lower lobar bronchi and segmental bronchi. The involving bronchi show smooth and nodular luminal narrowing. Right lower lobe is collapsed.

The bronchoscopy shows both bronchial mucosal wall thickening and nodularity. The orifice of right lower lobe superior segmental branchus is obstructed by extraluminal compression with edematous mucosa.

Brief Review

Amyloidosis is a multiorgan disease, localized amyloid deposits may affect single organ such as the kidney, bladder, or respiratory tract. Tracheobronchial amyloidosis is rare, but it is the most frequent form of primary pulmonary amyloidosis. The disease is chracterized by amyloid deposits within the submucosa and muscle of the tracheobronchial tree occurring as an isolated muscle or diffuse infiltration of the airway wall.
Patients are often asymptomatic but can demonstrate hemoptysis, striodor, cough, hoarseness, or wheezing. Men are affected more than women, and the mean age at presentation is 55-60 years.
The characteristic CT findings are circumferential wall thickening with calcification of the trachea and central bronchial tree with substantial narrowing of the main, lobar, and segmental bronchi. Although the CT findings of tracheobronchial amyloidosis are relatively specific to this disease, other tracheal diseases should be differentiated.
These include
1) tracheobronchopathia osteochondroplastica: thickening of the tracheal wall that spares the posterior tracheal membrane,
with irregular calcifications protruding into the lumen
2) relapsing polychondritis : calcification of the tracheobronchial tree, but limited to the cartilaginous ring

References

1.Bruce A. Urban, Elliot K. Fishman, CT Evaluation of Amyloidosis; spectrum of disease Radiographics
1993:13;1295- 1308
2.H.A.Pickford, S.J.Swensen, J.P.Utz Thoracic Cross-Sectional imaging of amyloidosis AJR ;168: 351-355,
February 1997
3.Tatsuya Yamamoto, Masayuki Maeda, Primary diffuse tracheobronchial amyloidosis. J of thoracic imaging,
2001:16:177-180
4. Christos S.Georgiades, Edward G.Neyman , Cross-sectional imaging of amyloidosis. J of computer
assisted tomography 2002:26(6):1035-1041

Keywords

Lung, Metabolic and storage lung disesae,