Pulmonary embolism is an unusual complication of Behçet’s disease (BD). Our aim study is to analyse epidemiological, physiopathological and evolutive aspects of this condition.

From 1998 to 2013, 10 cases of pulmonary embolism were found amongst BD patients in the department of internal medicine of Hedi Chaker Hospital. All these patients fulfilled International Study Group criteria for BD.

10 patients (all men, mean aged at 29.9±9.08 years) were diagnosed as having pulmonary embolism. This was inaugural in 7 cases. Peripheral venous thrombosis was present in 50% of patients; cardiac thrombosis was presented in 2 cases and pulmonary aneurysm in 2 patients. Pulmonary infarction has been noted in 3 cases. Pulmonary artery pressure may be elevated, and may indicate a poor prognosis. Mediastinal lymphadenopathy and mild pleural and pericardial effusions was also observed in 1 case. Protein C, protein S, antithrombin III and homocysteinemia levels were normal in all cases. One patient was positive for IgG anticardiolipin antibody. All our patients were treated successfully by anticoagulation therapy combined with high dose prednisone, colchicine and intravenous cyclophosphamide in 2 patients. After a mean follow-up of 106± 49 months, 4 patients had a recurrence of pulmonary embolism and only 1 patient was dead.

Pulmonary embolism is one of the severe and worst prognostic manifestations of the BD. Our knowledge about pulmonary complications of Behçet’s disease continues to evolve, but we need controlled trials for the management of the disease. The main goal should be to elucidate the pathogenesis and standardize the management according to the underlying pathologic process.