Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Pulmonary Amyloidosis, nodular type
- Radiologic Findings
- Chest radiograph shows multiple bilateral calcified and non calcified lung nodules. In addition, there is extensive reticulation in both lungs.
Axial non-contrast chest CT scans show multiple nodules in both lungs and some of them demonstrated cavitary changes. Dense calcifications or ossification in some nodules are seen. A combination of multifocal bronchiectasis with wall thickening and cysts is also seen in the lungs.
- Brief Review
- Amyloidosis is a heterogeneous group of diseases characterized by extracellular deposition of insoluble fibrillar proteins. The two most common forms are light-chain (AL) and reactive (AA) amyloidosis. The spectrum of pulmonary amyloidosis includes laryngeal, tracheobronchial, parenchymal and mediastinal lymph node disease. Pulmonary amyloidosis secondary to Sjogren’s syndrome is rare but presents with pulmonary nodules. The nodules usually are concentrated in the lower lobes and are typically subpleural or peripheral (1). They vary in size and shape with calcification or ossification is seen in up to 50% of cases.
In rare instances, nodular amyloid deposits may be associated with cysts, but their relationship is unclear. Proposed mechanisms include air trapping and destruction of alveolar walls by amyloid deposition and subsequent ischemia (2).
Sjogren syndrome, amyloid deposition, and lymphocytic interstitial pneumonia can occur together, and the cysts may then be a manifestation of lymphocytic interstitial pneumonia.
Amyloidosis is diagnosed on the basis of apple-green birefringence under a polarized light microscope after staining with Congo red, and the presence of rigid, non-branching fibrils, 7.5–10 nm in diameter, on electron microscopy.
- Please refer to
Case 277, Case 379, Case 608, Case 786, -
- References
- 1. Gillmore JD, Hawkins PN. Amyloidosis and the respiratory tract. Thorax 1999;54:444–51.
2. Ohdama S, Akagawa S, et al. Primary diffuse alveolar septal amyloidosis with multiple cysts and calcification. Eur Respir J 1996;9:1569–71.
- Keywords
- Lung, Metabolic and storage lung disesae,