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Fibrosing mediastinitis

Imaging findings

Chest CT in a 19-year-old female with ulcerative colitis shows progressive, bilateral, severe stenosis of the left main pulmonary artery and right upper lobe pulmonary artery from non-calcified infiltrative mediastinal soft tissue, leading to complete lack of perfusion to the left lung on VQ scan, with sparing of the pulmonary veins.

Key takeaways

Fibrosing mediastinitis classically manifests as a calcified hilomediastinal mass compressing the airways and blood vessels. In rare instances, it can present as a non-calcified soft tissue infiltrative process causing bilateral main pulmonary artery stenosis, mimicking Takayasu arteritis or IgG4-related disease.

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