[Clinical analysis of Behçet disease associated with pulmonary vascular disease].

To investigate the clinical features, therapy and prognosis of Behcet's disease(BD) complicated with pulmonary vascular diseases (PVD). The clinical manifestation, hemodynamics, pulmonary artery angiographic findings, therapy and prognosis of PVD in BD in Shanghai Pulmonary Hospital from January 2009 to August 2016 were analyzed retrospectively. Seven patients with average of (37±20) years were included. The ratio of male . female was 6∶1. Vascular involvement included pulmonary hypertension (PH) (4/7), pulmonary embolism (PE) (3/7) and pulmonary artery aneurysms (PAA) (3/7). All patients received immunosuppressive agents and all PH patients had therapy of PH target drugs. Three out of 4 PE patients underwent anticoagulant therapy. PVD is a rare complication of BD. The common clinical manifestations are PH, PE and PAA. Management of immunosuppressive agents may improve the prognosis and should be recommended.