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

ABSTRACT

Objective: To investigate the clinical features, therapy and prognosis of Behcet's disease(BD) complicated with pulmonary vascular diseases (PVD). Methods: 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. Results: Seven patients with average of (37±20) years were included. The ratio of male vs. 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. Conclusion: 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.