Scleroderma and failed response to alefacept.

conditions must also be considered in the differential diagnosis. Arterial aneurysmal rupture is the most common cause of death in Behçet’s disease [3]. Rupture may occur in 60% of arterial aneurysms and has a varying range of fatal outcome [7]. According to Urayama et al., the mortality rate in patients with arterial aneurysm is about 60% [9]. Furthermore, 50% of patients with pulmonary artery aneurysm, which is the form occurring most often, die after 10 months of haemoptysis [10]. Routine examination for vascular involvement includes palpation of the peripheral arteries and superficial veins, detecting impaired arterial perfusion and looking for evidence of superficial thrombophlebitis or deep-vein thrombosis, such as superior or inferior vena cava occlusion. The presenting symptoms are abdominal pain, abdominal distension and bleeding into the peritoneum, intestine and pancreatic duct. Imaging studies for all vascular involvement are plain abdominal X-ray, ultrasonography, Doppler ultrasonography, computed tomography with contrast, magnetic resonance imaging, magnetic resonance angiography, nuclear scanning, and digital subtraction angiography and venography. Medical treatment is mainly with immunosuppressive drugs (chlorambucil, cyclophosphamide, azathioprine and cyclosporin) and corticosteroids in suitable patients. Surgical therapy is indicated where there are non-regressive occlusions despite sufficient medical therapy, or large and ruptured aneurysms, or lesions resistant to medical agents [3, 8]. Because of the considerable tendency to rupture, surgical treatment should not be delayed in large aneurysms. To our knowledge, our case is the first splenic artery aneurysm and its rupture caused by Behçet’s disease reported in the literature. The patient was treated successfully and recovered completely. Arterial aneurysms are the major causes of death in Behçet’s disease. For this reason, patients with Behçet’s disease require close monitoring and routine examination for vascular involvement.