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Cystic pulmonary amyloidosis

Imaging findings

Chest CT shows multiple bilateral, thin-walled cystic lesions, some containing residual tissue or internal septations, associated with parenchymal nodular opacities showing progressive growth and calcification/ossification. FDG-PET shows localized avidity, and biopsy confirmed positive Congo red staining for amyloid.

Key takeaways

Cystic pulmonary amyloidosis is a rare localized protein deposition disease that can present as multiple thin-walled cysts and nodular opacities, sometimes mimicking lymphocytic interstitial pneumonia or Sjogren's syndrome. The cysts form due to degradation of extracellular matrix by matrix metalloproteinases associated with the amyloid deposits.

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