CHEST Guidelines-Emphysematous-Lung-Lesions-Caused-by-Perivascular-

Emphysematous-Lung-Lesions-Caused-by-Perivascular-

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This document presents a case study on a rare presentation of systemic amyloid light-chain (AL) amyloidosis. Amyloidosis is a condition where abnormal protein deposits accumulate in the body's tissues and organs. In this case, a 75-year-old woman with a history of lymphoplasmacytic lymphoma (LPL) presented with severe exertional dyspnea and emphysematous changes—a form of lung damage typically characterized by airspace enlargement on her chest CT scan. Although nodular opacities and diffuse interstitial infiltrates are common radiographic manifestations of pulmonary amyloidosis, the presence of emphysematous lesions, especially in the context of AL amyloidosis, is exceedingly rare with only one prior report.<br /><br />The chest CT findings revealed mosaic attenuation in the upper and mid-lung areas and emphysematous changes predominantly in the lower lungs. A surgical lung biopsy was pursued due to the diagnostic uncertainty, revealing perivascular and interstitial (alveolar-septal) amyloid deposition. The condition was treated with Ibrutinib, targeting underlying LPL and systemic amyloidosis, leading to symptomatic improvement.<br /><br />The case highlights potential variability in pulmonary amyloidosis presentation and emphasizes the necessity of considering emphysematous changes as a diagnostic possibility when faced with similar radiographic findings and clinical symptoms. It further underscores the importance of histopathology correlated with imaging to understand the deposition patterns in different lung areas.<br /><br />This case study contributes important insights to the limited body of knowledge on the unique presentations of pulmonary involvement in amyloidosis, driving home the need for clinicians to broaden their differential diagnosis in cases of unexplained dyspnea with atypical lung findings.

Keywords

systemic amyloidosis

light-chain amyloidosis

pulmonary involvement

lymphoplasmacytic lymphoma

emphysematous changes

mosaic attenuation

lung biopsy

Ibrutinib treatment

histopathology

dyspnea