Abstract Introduction Pulmonary artery aneurysm (PAA) is an uncommon but potentially life-threatening vascular complication of fibrosing mediastinitis. We describe a patient with a right pulmonary artery aneurysm and severe pulmonary artery stenosis due to fibrosing mediastinitis secondary to sarcoidosis. Case Summary A 68-year-old woman of mild intermittent asthma presented with a chronic dry cough for six months. She had initially been diagnosed with asthma and started on an inhaled corticosteroid and short-acting beta-agonist. A computed tomography (CT) of the chest without contrast demonstrated multiple bilateral perilymphatic pulmonary nodules with calcified mediastinal and hilar lymphadenopathy suggestive of granulomatous disease such as sarcoidosis. Pulmonary function testing revealed mixed obstructive and restrictive physiology with reduced diffusing capacity. Elevated soluble interleukin-2 receptor levels (2156 pg/ml) supported a diagnosis of sarcoidosis. Flexible bronchoscopy demonstrated a large, pulsatile vascular lesion in the right bronchus intermedius, with significant erythema and vascular congestion in the proximal airways. Cultures were negative for infectious etiology, and cytology was negative for malignancy. Follow-up CT angiography of the chest showed severe right interlobar pulmonary artery stenosis with proximal and distal aneurysmal dilation and endobronchial extension, attributed to extrinsic compression from calcified hilar lymph nodes, consistent with fibrosing mediastinitis secondary to sarcoidosis. The patient underwent right pulmonary angiography and successful endovascular stenting (7 mm × 15 mm VBX stent). Discussion This case illustrates a rare vascular complication of fibrosing mediastinitis secondary to sarcoidosis. Lymph node calcification and fibrosis may result in pulmonary artery stenosis with secondary aneurysmal remodeling. (1). Awareness of this entity and consideration of contrasted chest imaging are essential to prevent life-threatening hemoptysis and guide timely vascular intervention. Conclusion An endobronchial pulmonary artery aneurysm can lead to life-threatening hemoptysis. Early recognition and intervention of vascular complications of fibrosing mediastinitis are important to reduce morbidity and mortality. References1.Argueta F, Villafuerte D, Castaneda-Nerio J, Peters J, Restrepo C. Successful management of fibrosing mediastinitis with severe vascular compromise: Report of two cases and literature review. Respir Med Case Rep. 2020;29:100987. This abstract is funded by: None
Alachraf et al. (Fri,) studied this question.