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Granulomatous Fibrosing Mediastinitis (Presumed Histoplasmosis)

Imaging findings

Chest CT demonstrates a calcified mediastinal and bilateral hilar mass causing encasement and severe narrowing of the pulmonary arteries and veins. There is a chronic, large left-sided pleural effusion with associated pleural thickening and multiple parenchymal calcifications. Notably, severe obstruction of the left inferior pulmonary vein has led to the development of massive, tortuous pulmonary varices in the left lower lobe, representing collateral venous pathways secondary to pulmonary venous hypertension.

Key takeaways

Fibrosing mediastinitis is a rare, life-threatening complication characterized by an abnormal, progressive, invasive fibrotic reaction in the mediastinum, most commonly triggered by an immune-mediated hypersensitivity response to Histoplasma capsulatum. The disease typically targets soft mediastinal structures, leading to the compression and occlusion of the superior vena cava, pulmonary arteries, airways, and pulmonary veins. Left-sided pulmonary venous obstruction can mimic mitral stenosis or left heart failure, presenting as unilateral pulmonary venous hypertension, asymmetric pleural effusions, and large compensatory pulmonary varices.

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