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Systemic AL Amyloidosis with Pulmonary and Pleural Involvement

Imaging findings

A patient with systemic AL amyloidosis involving the kidney, liver, and heart presented with a spontaneous secondary pneumothorax and pleural effusion. Chest radiograph and CT showed extensive bilateral reticulonodular disease, right greater than left, with numerous nodules containing dense calcium/ossification, nodular thickening along the interlobular septa and subpleural regions and fissures, and a right pleural effusion; the pattern of irregular, branching, pleura-hugging calcified/ossified nodules closely mimicked diffuse pulmonary ossification (DPO).

Key takeaways

Pulmonary and pleural (septal) amyloidosis can produce extensive nodular disease with branching dystrophic calcification/ossification that is difficult to distinguish from idiopathic diffuse pulmonary ossification, and amyloidosis should be considered when this DPO-like pattern is seen, particularly with pleural involvement; a spontaneous pneumothorax from pulmonary amyloid involvement is an unusual but reported presentation.

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