Vascular and cardiac involvement

Abstract

As is true for other major organ involvement in Behçet syndrome (BS), vascular involvement is more common and runs a more severe course among males. It is a major cause of morbidity and mortality and has significant characteristic features that can be considered pathognomonic even without the concomitant presence of skin-mucosa lesions. It affects mostly veins, with a significant tendency for thrombosis and as a rule runs a relapsing course. As a result, patients tend to have several clusters of vascular involvement through their disease course. Vascular involvement either venous or arterial occurs diffusely in large segments through the vessel wall. We have now become aware of vein walls that may be thickened without overt vein disease among young patients with solo skin-mucosa lesions indicating that venous inflammation is the key feature in BS. The associated thrombus is strongly adherent to long segments of the wall with a low propensity for the development of emboli. Lower extremity deep vein thrombosis (LEDVT) is the earliest and most frequent manifestation, followed by vena cava inferior thrombosis. Dural sinuses and pulmonary arteries, structurally similar to veins, are also commonly involved, while pulmonary artery aneurysms are almost specific for BS. Despite intensive and early immunosuppressive treatment, mortality especially due to pulmonary artery involvement and Budd-Chiari syndrome is still increased. Additionally, the young male patient population with LEDVT alone is exposed to severe clinical and social consequences such as post-thrombotic syndrome and increased unemployment.