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Isolated pulmonary artery vasculitis is an uncommon cause of pulmonary artery aneurysm with very few reported cases in the literature. We hereby present the case of a 70-year-old man with occasional episodes of exertional chest discomfort. Our investigations revealed an expanding aneurysm of the main pulmonary artery extending to the proximal portion of the right branch. The patient successfully underwent replacement of the main pulmonary artery with a homograft. Histopathological examination revealed images of vasculitis with numerous multinucleated giant cells. The patient’s postoperative course was uneventful. Management of pulmonary artery aneurysm secondary to isolated pulmonary artery vasculitis is not well studied, and no clear guidelines currently exist in the literature.

Chest discomfort leading to the diagnosis of pulmonary artery aneurysm due to isolated main pulmonary arteritis involving giant cells: a case report