Vascular manifestations of Behcet's disease

Objective: To study the incidence of vascular disorders and to estimate their contribution to the severity and prognosis of Behcet's disease (BD). Subjects and methods. Ninety-five patients with evident BD, followed up at Research Institute of Rheumatology, Russian Academy of Medical Sciences, in the January 2006 to October 2009, were examined. Their mean age was 29,7 years; malefemale ratio was 3,7. The onset of the disease was chiefly at the age of 21-30 years in 36,8% of the patients.
Vascular pathology was evaluated by the following techniques: vascular duplex scanning; brain magnetic resonance imaging in the mode of venosinusography, contrast-enhancement chest computed tomography, chest and abdominal angiography. The data were statistically processed by the programs Statistica 6.0 (Statsoft, USA) and EpiInfo 5.0 recommended by the WHO. Parametric and non-parametric statistical methods were used.
Results. Vascular diseases were diagnosed in 26,3% of the patients. The venous bed was mainly involved (88%), the proportion of patients with arterial pathology was 12%. Vascular death occurred in 2 men aged 20 and 18 years due to pulmonary artery rupture aneurysm and brain sagittal sinus thrombosis.
Assessment of an association of vascular disorders with other clinical manifestations of BD revealed their correlation with erythema nodosum and epididymitis. The risk for vascular events in erythema nodosum was 5 times greater (OR=5,03; 95% CI 1,54-17,41), the risk was higher in men than that in women (OR=7,11; 95% CI 1,83-30,02 versus OR=2,15; 95% CI 0,16-61,38). The male vascular risk is associated with epididymitis (OR=6,71; 95% CI 1,25-39,89). In erythema nodosum concurrent with epididymitis, the relative vascular risk was increased up to 21; 95% CI 2,15-503,94.
Conclusion. Vascular disorders in BD were diagnosed in one fourth of the patients, mainly in young male patients. Severe thromboses with the development of chronic venous insignificance, Budd-Chiari syndrome, pulmonary and iliac artery aneurysms, and arterial thromboses were observed in male patients only. Vascular events were associated with erythema nodosum and epididymitis; in these concomitances, the vascular risk was substantially increased. Vascular death rates were 2,2%.

Behcet's disease, venous and arterial thromboses, arterial aneurysms

1. <div><p>Aлекберова З.С. Болезнь Бехчета. М., 2007;97с.</p><p>Behcet's H. Über rezidivierende aphthö se, durch ein Virus verursachte Geschwii re am Mund, am Auge und an den Genitalien. Dermatol Wochenschr 1937;105:1152-5.</p><p>Jensen T. Ulcerous hemorrhagic colitis associated with Behcet's syndrome. Ugeskrift För Laeger 1941b;106:176-80.</p><p>Berlin C. Behcet's syndrome with involvement of central nervous system. Report of a case, with necropsy, of lesions of the mouth, genitalia and eyes; review of the literature. Arch Dermatol Syph (Chicago) 1944;49:227-33.</p><p>Sugioka I., Ohe H., Tanaka T. Two cases of hip joint deformation associated with Behcet's syndrome. Orthop Surg Traumatol 1966;15:131.</p><p>Zouboulis C, Keitel W. A Historical Review of early descriptions of Adamantiades-Behcet's disease. J Invest Dermatol 2002;119:201-5.</p><p>International Study Group for Behcet's Disease. Criteria for diagnosis of Behcet's disease. Lancet 1990;335:1078-80.</p><p>Zouboulis C, Vaiopoulos G, Macromichelakis N. et al. Onset signs, clinical course, prognosis, treatment and outcome of adult patients with Adamantiades -Behcet's disease in Greece. Clin Exp Rheum 2003;21(Suppl. 30):19-26.</p><p>Yazici H., Esen F. Mortality in Behcet's syndrome. Clin Exp Rheumatol 2008;26:138-40.</p><p>Kaklamani V., Vaiopoulos G., Kaklamanis P. Behcet's disease. Semin Arthritis Rheum 1998;27:197-217.</p><p>Koc Y., Gullu I., Akpek G. et al. Vascular involvement in Behcet's disease. J Rheumatol 1992;19:402-10.</p><p>Gurler A., Boyvat A, Tursen U. Clinical manifestations of Behcet's disease: an analysis of 2147 patients. Yonsei Med J 1997;38:423-7.</p><p>Jesus H. de, Rosa M., Queiroz M.V. Vascular involvement in Behcet's disease. Ananalysis of twelve cases. Clin Rheum 1997;16:220-1.</p><p>Zouboulis Ch., Kötter I., Djawari D. et al. Epidemiological features of Adamantiades-Behcet's disease in Germany and in Europe. Yonsei Med J 1997;38:411-22.</p><p>Benamour S., Zeroual B., Bennis R. et al. Mala-die de Behcet-316 cases. La Presse Medicale 1990;19:1485-9.</p><p>Shimizu T., Ehrlich G., Inaba G. et al. Behcet's disease (Behcet's syndrome). Sem Arthritis Rheum 1979;8(4):223-60.</p><p>Hamza M. Angio Behcet. Paris: Elsevier Science Publishers B.V., 1993;7.</p><p>Yazici H., Basaran G., Hamuryan V. et al. The ten-year mortality in Behcet's syndrome. Br J Rheumatol 1996;35:139-41.</p><p>Ames P., Steuer A., Pap A. et al. Thrombosis in Behcet's disease: a retrospective survey from a single UK center. Rheumatology 2001;40:652-5.</p><p>Zouboulis Ch. Epidemiology of Adamantiades-Behcet's disease. BD News 2001;2:3-4.</p><p>Kaklamani V., Kaklamanis P. Treatment of Behcet's disease: an update. Sem Arthr Rheum 2001;30:299-312.</p><p>Tugal-Tutkun I., ?nal S., Altan Yayciolglu R. Uveitis in Behcet's disease: an analysis of 880 patients. Am J Ophthalmol 2004;138:373-80.</p><p>Al-Otaibi L., Porter S., Poate T. Behcet's disease: A review. J Dent Res 2005;84:209.</p><p>Cho Y., Jung J., Lee K. et al. Clinical features of patients with Behcet's disease and epididymitis. J Urol 2003;170:1231.</p><p>Kuzu M., Ozaslan C., Köksoy C. et al. Vascular involvement in Behcet's disease: 8-year audit. World J Surg 1994;18(6):948-54.</p><p>Behcet's disease Research Committee of Japan. Behcet's disease: guide to diagnosis of Behcet's disease. Jpn J Opht 1974;18:291-4.</p><p>Houman H., Lamloum M., Ben Ghorbel I. et al. Vena cava thrombosis in Behcet's disease. Analysis of a series of 10 cases. Ann Med Intern 1999;150:587-90.</p><p>Pasic M., Senning A., von Segesser L. et al. Transcaval liver resection with hepatoatrial anastomosis for treatment of patients with Budd-Chiari syndrome. J Thorac Cardiovasc Surg 1993;106:275-82.</p><p>Wang Z., Zhu Y., Wang S. et al. Recognition and management of Budd-Chiari syndrome: report of one hundred cases. J Vasc Surg 1989;10:149-56.</p><p>Orloff M.J., Orloff M.S., Daily P. Longterm results of treatment of Budd-Chiari syndrome with portal decompression. Arch Surg 1992;127:1182-8.</p><p>Uskudar O., Akdogan M., Sasmaz N. et al. Etiology and portal vein thrombosis in Budd-Chiari syndrome. World J Gastroenterol 2008;14(8):2858-62.</p><p>Maruyama R., Kumaki T., Miyashita M. et al. Case of Behcet's syndrome accompanied by Budd-Chiari syndrome. Naika 1967;19(5):959-64.</p><p>Temizoz O., Genchellac H., Yekeler E. CT-angiographic demonstration of hepatic collateral pathways due to superior vena cava obstruction in Behcet's disease. Diagn Interv Radiol 2009 Oct., doi: 10.4261/1305-3825.DIR.2174-08.2.</p><p>Siva A., Saip S. The spectrum of nervous system involvement in Behcet's syndrome and its differential diagnosis. J Neurol 2009;256:513-29.</p><p>Wechsler B., Vidailhet M., Piette J. et al. Cerebral venous thrombosis in Behcet's disease: clinical study and long-term follow-up of 25 cases. Neurology 1992;42:614-8.</p><p>Mishima Y., Ishikawa K., Kawase S. Behcet's syndrome with aneurysm (abstr). In: Proceedings of the Japanese Circulation Society, Tokyo, 1960. Jpn Cir J 1961;25:1211.</p><p>Uzun O., Akpolat T., Erkan L. Pulmonary vasculitis in Behcet's Disease. A cumulative analysis. Chest 2005;127:2243-53.</p><p>Erkan F., Cavdar T. Pulmonary vasculitis in Behcet's disease. Am Rev Respir Dis 1992;146:232-9.</p><p>Erkan F., Gul A., Tasali E. Pulmonary manifestations of Behcet's disease. Thorax 2001;56:572-8.</p><p>Hiller N., Lieberman S., Chajek-Shaul T. et al. Thoracic manifestations of Behcet's disease at C T. Radio Graphics 2004;24:801-8.</p><p>Le T., Bletry O., Wcchsler B. et al. Arterial manifestations in Behcet's disease: fifteen cases in a series of 250 patients. Behcet's disease basic and clinical aspects by Desmond O'Duffy and Emre Kokmen-Mayo Clinic, Rochester, Minnesota: Marcel Dekker, Inc., 1991;21:145-53.</p><p>Park J., Han M., Bettmann M. Arterial manifestations of Behcet's disease. AJR 1984;143:821-5.</p><p>Raz I., Okon E., Chajek-Shaul T. Pulmonary manifestations in Behcet's syndrome. Chest 1989;95:585-9.</p><p>Santana A., Antunes T., Barros J. et al. Pulmonary involvement in Behcet's disease: a positive single-center experience with the use of immunosuppressive therapy. J Bras Pneumol 2008;34(6):362-6.</p><p>Hamuryudan V., Yurdakul S., Moral F. et al. Pulmonary artery aneurysms in Behсet's syndrome: a report of 24 cases. Br J Rheumatol 1994;33:48-51.</p><p>Chalazonitis A., Lachanis S., Mitseas P. et al. Hughes-Stovin syndrome: a case report and review of the literature. Cases J 2009;2:98.</p><p>Espinosa G., Cervera R., Reverter J. et al. Vascular involvement in Behcet's disease. Isr Med Assoc J 2002;4(8):614-6.</p><p>Leiba M., Seligsohn U., Sidi Y. et al. Thrombophilic factors are not leading cause of thrombosis in Behcet's disease. Ann Rheum Dis 2004;63:1445-9.</p><p>Ko S., Kang S., Lee S. et al. Massive thrombosis after central venous catheterization in a patient with previously undiagnosed Behcet's disease. J Korean Med Sci 2001;16:814-6.</p><p>Kingston M., Ratcliffe J., Alltree M. et al. Aneurysm after arterial puncture in Behcet's disease. Br Med J 1979;30,1(6180):1766-7.</p><p>Tunaci M., Ozkorkmaz B., Tunaci A. et al. CT findings of pulmonary artery aneurysms during treatment for Behcet's disease. Am J Roentgenol 1999;172:729-33.</p><p>Akpolat T., Danaci M., Belet U. et al. MR imaging and MR angiography in vascular Behcet's disease. Magn Reson Imaging 2000;18:1089-96.</p></div><br />