CTICasesFibrosing Hilaritis Mimicking CTEPH
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Imaging findings
A ventilation-perfusion scan shows a matched/unmatched perfusion defect involving the entire right lung, leading to a misdiagnosis of chronic thromboembolic pulmonary hypertension (CTEPH). Contrast-enhanced computed tomography of the chest reveals complete occlusion of the right interlobar pulmonary artery and the right superior pulmonary vein, accompanied by narrowing of the bronchus intermedius and a calcified soft-tissue mass in the right hilum. Marked systemic collaterals from hypertrophied bronchial, internal mammary, and inferior phrenic arteries are present, along with calcified granulomas in the spleen.
Key takeaways
Fibrosing hilaritis (a localized form of fibrosing mediastinitis, typically secondary to old histoplasmosis) is a classic mimic of CTEPH on VQ scans and CT. Unlike CTEPH, which typically spares the pulmonary veins and airways, fibrosing hilaritis is characterized by a calcified hilar mass that concomitantly constricts pulmonary arteries, pulmonary veins, and bronchi, often with splenic calcified granulomas as a clue to prior granulomatous infection.
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