General ReviewMajor Arterial Involvement and Review of Behcet's Disease
Introduction
Behcet's disease is an autoimmune multisystemic disorder based on vasculitis. Characteristicly the disease involves three systems, so-called “triple symptom complex”: the eye, mouth, and genitalia.1 The major manifestations are recurrent oral and genital aphtous ulcerations. Skin lesions include erythema nodosum-like eruptions, subcutaneuos thrombophlebitis, and hyperirritablity of the skin. Eye lesions consist of recurrent hypopyon iritis or iridocyclitis and chorioretinitis. It is also a multisystemic chronic inflammatory disease and may involve neurologic, cardiovascular, pulmonary, and musculoskeletal systems.
Vascular manifestations, although rare, may occur. Incidence of vascular involvement ranges between 7% and 29%.2 The disease affects both arteries and veins with various lesions ranging from superficial thrombophlebitis to occlusive lesions of vena cava, as well as stenosis and aneurysms of the aortic arch, thoracic and abdominal aorta, and peripheral arteries. Although venous thrombosis is the most frequent vascular complication, aneurysms and subsequent rupture of the large arteries constitute the major cause of mortality. Occlusive lesions usually occur in large arteries such as subclavian, pulmonary, and femoropopliteal arteries,3 but smaller arteries can also be involved. Occasional involvement of the coronary arteries leading to myocardial infarction has also been reported.4
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Materials and Methods
The data of 31 patients with the diagnosis of vasculo-Behcet's disease who were treated in Istanbul University, Istanbul Medical Faculty, and Department of Cardiovascular Surgery were reviewed retrospectively. Our series showed a male predominance (three female and 28 male patients). The mean age of patients was 41. Smoking was present as a cofactor in 25 patients. The mean duration of the Behcet's disease and of vascular involvement were 5 and 3 years, respectively. We examined all patients by
Results
Among 15 patients with venous involvement, there were 10 with superficial lower extremity thrombophlebitis, three with deep vein thrombosis of the lower extremity, and two with superior vena cava thrombosis. All of these patients received intravenous heparin therapy. All the thrombophlebitis and superior vena cava thrombosis cases were treated. However, among the three deep vein thrombosis patients, only one of them was treated and the remaining two resulted in postthrombophlebitic syndrome.
Ten
Discussion and Conclusions
Behcet's disease is an autoimmune systemic vasculitis that involves vessels of any diameter and causes various repetitive clinical manifestations, such as oral and genital ulcerations, uveitis, and typical skin lesions with positive pathergy test (hyperreactivity of skin to sterile needle prick). Hulusi Behcet first reported the disease in 1924 (named with his name as Behcet's disease in 1937) and since then, many clinical reports followed, and the exact features of the disease gradually became
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