This review article discusses three topics related to pulmonary vascular disease: 1) pulmonary vascular changes associated with portal hypertension, 2) ANCA-associated pulmonary vasculitis, and 3) Takayasu's arteritis. Hepatopulmonary syndrome and pulmonary hypertension have recently been reported as pulmonary vascular changes accompanied with portal hypertension. Endogenous vasoactive agents that reach the pulmonary circulation through porto-systemic shunt vessels are thought to contribute to these vascular changes. In ANCA-associated vasculitis, hemorrhage, interstitial pneumonitis, and nodular lesions are common manifestations in the lung. In Takayasu's arteritis, CT occasionally demonstrates mosaic attenuation owing to pulmonary arteritis and peripheral reticulolinear changes probably due to thromboembolism.