Major vascular involvement in Behçet’s disease: a retrospective study of 796 patients

Abstract

Behçet's disease (BD) is a multi-systemic inflammatory disorder which can affect all types and sizes of blood vessels. This study aims to evaluate the prevalence and characteristics of vascular involvement in BD. Among 796 patients diagnosed with BD, 102 patients (81 male, 21 female) with vascular involvement were included, whose detailed clinical characteristics were recorded. The diagnosis of vascular lesions was made on clinical signs, by Doppler ultrasonography, and/or angiography using computed tomographic or magnetic resonance techniques. Vascular involvement occurred in 12.8 % of BD patients. Male to female ratio was 3.86:1. Mean age at onset of vascular involvement was 29.5 ± 11.3 years. Vascular lesion was the initial sign of BD in 28 patients, accounting for 27.5 %. Of 102 BD patients with vascular involvement, 72 had venous lesions (70.6 %) and 56 had arterial lesions (54.9 %), among which 26 (25.5 %) patients had both venous and arterial involvements. Female BD patients were more often involved with arterial lesions, whereas male BD patients developed venous lesions more often than females, P = 0.000. The most common type of vascular involvement was deep venous thrombosis in lower extremities (n = 49), other affected venous sites including inferior vena cava, superior vena cava, and cerebral venous. The prominent type of arterial lesions was dilatation (n = 25, including 24 cases of aneurysms); other types included eight cases of occlusion and 23 cases of stenosis. The main locations of arterial lesions were the aorta (n = 19), lower extremity arteries (n = 15), pulmonary arteries (n = 13), coronary arteries (n = 5), and subclavian arteries (n = 5). Compared with those without vascular lesions, ocular involvement, genital ulcers, and arthritis were significantly less frequent among patients with vasculo-BD (23.5 vs 35.2 %, P = 0.024; 54.9 vs 76.5 %, P = 0.000; 19.6 vs 30.5 %, P = 0.026), whereas a higher frequency of cardiac involvement was found in vasculo-BD patients (20.6 vs 3.6 %, P = 0.000). Vascular involvement is a complication in BD patients. This study illustrated that venous lesions are more frequently involved than arterial lesions. Vascular lesions correlated with a high frequency of cardiac involvement and a low incidence of ocular lesions, genital ulcers, and arthritis.

Venous thromboembolism in systemic autoimmune diseases: A narrative review with emphasis on primary systemic vasculitides

“…56 The frequency of vascular involvement in BD in various studies ranges from 1.8% to 51.6%. 57 Venous disease in BD can manifest as deep vein thrombosis, superficial thrombophlebitis, cerebral venous sinus thrombosis, vena cava thrombosis, Budd-Chiari syndrome, or pulmonary artery thrombosis. 57 Venous involvement in BD also varies by the series studied.…”

“…57 Venous disease in BD can manifest as deep vein thrombosis, superficial thrombophlebitis, cerebral venous sinus thrombosis, vena cava thrombosis, Budd-Chiari syndrome, or pulmonary artery thrombosis. 57 Venous involvement in BD also varies by the series studied. Of 6500 Iranian patients with BD, 1.1% had vena cava thrombosis.…”

Endovascular treatment of aortoiliac occlusion in a young patient with Behçet’s disease

“…Other reported arterial lesions include subclavian, common carotid, coronary, brachial, radial, ulnar, common iliac, external iliac, tibial, renal, cerebral, axillary, and splenic arteries. [1,2,[5][6][7] Our case had a history of deep vein thrombosis five years ago and claudication thereafter.…”

Patients with Behcet’s Disease and Cardiovascular Risk Factors are at Risk for Early Cardiovascular and Renal Disease

“…Superficial and deep lower limb thrombosis are the most frequent venous complications but one third of venous thrombosis concerns large vessels (such as cerebral venous thrombosis, pulmonary embolism, and inferior or superior vena cava). Arterial complications include aneurysms and occlusions/stenosis [3].…”

“…Vascular involvement also occurs less often, but has a high mortality rate of 20% [2]. Both arterial and venous involvement have been reported and may involve vasculitis of all types of blood vessels [3]. Complications of vascular origin in BD patients, appearing in 7% to 46% of patients, include aortic and pulmonary artery aneurysms, thrombophlebitis, and venous and arterial occlusions [1,2].…”