[Cardio-pulmonary disorders associated with collagen vascular disease]

Among the cardio-pulmonary disorders associated with collagen vascular disease (CVD), pulmonary arterial hypertension (PAH) is recognized as most serious problem. Because PAH is difficult to manage and its prognosis is terribly poor. Thus, early diagnosis of PAH in CVD is important. Since PAH patients associated with CVD are often asymptomatic in their early stage, screening with echocardiography is needed. The degree of inflammation causing PAH through endothelial injury is closely associated with CVD, and thus, the improvement of PAH may be achieved by immunosuppressant, especially in its early stage. Once the diagnosis of PAH is confirmed, treatment with oral vasodilators should be started as soon as possible. If oral vasodilators prove inadequate, intravenous epoprostenol should be administered without hesitating.