CTICasesFibrosing Hilaritis and Unilateral Fibrothorax due to Histoplasmosis
Imaging findings
Contrast-enhanced chest CT demonstrates a small right hemithorax with severe calcified fibrothorax, splenic calcified granulomas, and extensive calcified hilar lymphadenopathy with soft-tissue proliferation. The soft tissue completely occludes the right interlobar pulmonary artery and narrows the middle lobe bronchus, consistent with fibrosing hilaritis.
Key takeaways
Fibrosing hilaritis (or fibrosing mediastinitis) is an uncommon, exaggerated immune-mediated fibrotic reaction to past Histoplasma capsulatum infection that causes progressive narrowing and occlusion of the hilar airways, pulmonary arteries, and veins. When associated with unilateral fibrothorax, it may reflect historical, localized granulomatous pleuritis or empyema.
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