Pulmonary artery involvement as first manifestation in three cases of Takayasu arteritis

Although the pulmonary artery is often involved in Takayasu arteritis, only a few cases have been reported in which pulmonary artery involvement occurred as the initial clinical manifestation. Based upon history, physical findings, laboratory data, and radiographic studies, we diagnosed Takayasu arteritis in three young females who had pulmonary artery occlusion without apparent lesions in the aortic arch and its main branches. Major symptoms and signs encountered were persistent mild or moderate chest pain, pleural effusion, and an episode of hemoptysis. Pulmonary artery occlusions were confirmed by angiography and perfusion studies. Steroid treatment relieved the symptoms in two cases, but the 3rd case required additional therapy with methotrexate for the suppression of disease activity. We emphasize the importance of considering Takayasu arteritis in cases with the above-mentioned signs and symptoms and pulmonary artery occlusion, despite the absence of aortic lesions.