CTICasesPrimary Pulmonary Amyloidosis (Alveolar Septal Pattern)
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Imaging findings
Chest radiograph initially shows increased peribronchovascular opacities predominantly in the lower lobes, potentially with a pleural effusion or thickening. Subsequent CT scans show progressive, extensive lymphadenopathy, areas of consolidation, significant septal thickening, peribronchial wall thickening, and increasing attenuation within the consolidation, which evolved into diffuse metaplastic bone formation. The imaging pattern suggests an alveolar septal or interstitial pattern of amyloidosis, rather than nodular.
Key takeaways
Primary pulmonary amyloidosis can manifest as an alveolar septal or interstitial pattern characterized by septal and peribronchial thickening, often with confluent areas of consolidation. Progressive disease can lead to extensive pulmonary ossification (metaplastic bone formation). This form is distinct from nodular amyloidosis, which can be associated with cysts. Histopathology often describes involvement of alveolar septa and vessels microscopically, which correlates with the imaging findings of septal thickening and consolidation. This disease can cause severe dyspnea and often has a poor prognosis.
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