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Systemic AL Amyloidosis

Imaging findings

CT chest shows pleural effusions and a highly asymmetric pattern of thick septal lines, predominant on the right. Core biopsy of the lung shows positive Congo red staining of the interstitium, and the patient has an associated monoclonal gammopathy and amyloid cardiomyopathy.

Key takeaways

Pulmonary AL amyloidosis can present with a diffuse interstitial septal pattern, mimicking pulmonary edema or lymphangitic carcinomatosis. The asymmetric distribution and presence of associated cardiomyopathy, pleural effusions, and monoclonal gammopathy are key diagnostic clues.

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