The Evaluation of Coagulation Parameters and Vessel Involvement in Behcet’ s Disease. A Clinical Experience of Behcet’ s Disease: Study of 152 Cases
Behcet´s disease (BD) is accepted as a systemic vasculitis. Vasculitis is observed predominantly on the venous system. Vessel involvement is frequently seen in males. This study was planned retrospectively evaluate demographic features, clinical features, vessel involvements in BD. Furthermore, we aimed to prospectively compare consecutively chosen patients with and without thrombosis and healthy volunteers in terms of their biochemical, immunological, coagulation parameters. One hundred fifty-two Behcet´s patients were retrospectively evaluated. Blood samples were collected from 52 consecutively chosen patients and 41 healthy subjects. Papulopustular skin lesions, eye involvement and venous lesions were detected frequent in males. In terms of evaluated parameters (biochemical parameters, coagulation parameters, C-reactive protein, erythrocyte sedimentation rate, anticardiolipin antibodies, antinuclear antibody positivity) was not found a significant difference among groups (patients without thrombosis, healthy control subjects, patients with thrombosis). We detected statistically significant difference in terms of factor V levels between patient and control group. The tendency to thrombosis in patients with BD is well known. The endothelial lesion, increased procoagulant activity, hypofibrinolysis were found to be responsible from these events. In our study, there was no significant difference in terms of coagulation parameters between the patients without and with thrombosis.
Nakae K, Masaki F, Hashimoto T, et al. Recent epidemiological features of Behçet’s disease in Japan. In: Godeau P, Wechsler B, eds. Behçet’s disease. Proceedings of the 6th International Conference on Behçet’s Disease. Amsterdam: Elsevier 1993:145-151.
Shimizu T, Ehrlich GE, Inaba G, Hayashi K. Behçet disease (Behçet syndrome). Semin Arthritis Rheum 1979;8:223-260.
Seyahi E, Yurdakul S. Behçet’s Syndrome and Thrombosis. Mediterr J Hematol Infect Dis 2011;3(1):e2011026. Epub 2011 Jul 8.e 2011026, DOI 10.4084/MJHID.2011.026.
Salvarani C, Pipitone N, Catanoso MG, Cimino L, Tumiati B, Macchioni P, Bajocchi G, Olivieri I, Boiardi L. Epidemiology and clinical course of Behçet’s disease in the Reggio Emilia area of Northern Italy: a seventeen-year population-based study. Arthritis Rheum 2007;57:171-8.
Yazıcı H, Tüzün Y, Pazarlı H, Yurdakul S, Ozyazgan Y, Ozdoğan H, Serdaroğlu S, Ersanli M, Ulkü BY, MüftüoğluAU. Influence of age of onset and patients’s sex on the prevalence and severity of manifestations of Behçet’s syndrome. Ann Rheum Dis 1984;43(6):783-9.
Zouboulis CC, Kötter I, Djawari D, Kirch W, Kohl PK, Ochsendorf FR, Keitel W Stadler R, Wollina U, Proksch E, Söhnchen R, Weber H, Gollnick HP, Hölzle E, Fritz K,Licht T, Orfanos CE. Epidemiological features of Adamantiades-Behçet’s disease in Germany and in Europe. Yonsei Med J 1997;38(6):411-422.
Kaklamani VG, Vaiopoulos G, Kaklamanis PG. Behçet’s disease. Semin Arthritis Rheum 1998;27:197-217.
O’Neil TW, Rigby AS, McHugh S, Silman AJ, Barnes C. Regional differences in clinical manifestations ofBehçet’s Disease. In Godeau P, Wechsler B, eds. Behçet’s disease. Proceeding of the 6th International Conference on Behçet’s Disease. Amsterdam:Elsevier 1993:145-51.
Serdaroğlu P, Yazici H, Ozdemir C, Yurdakul S, Bahar S, Aktin E. Neurologic involvement in Behçet's syndrome. A prospective study. Arch Neurol 1989;46:265-9.
Ohno S, Ohguchi M, Hirose S, Matsuda H, Wakisaka A, Aizawa M. Close association of HLA-Bw51 with Behçet’s disease. Arch Ophthalmol 1982;100:1455-8.
Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. Lancet 1990;335:1078-80.
O'Neill TW, Rigby AS, Silman AJ, Barnes C. Validation of the International Study Group criteria for Behçet's disease. Br J Rheumatol 1994;33:115-7.
O'Duffy JD, Goldstein NP. Neurologic involvement in seven patients with Behçet's disease. Am J Med 1976;61:170-8.
Dilşen N, Koniçe M, Aral O, Ocal L, Demiryont M. Thecharacteristics and significance of cardiovascular involvement in our 487 cases of Behçet’s disease. VIth EULAR SYMPOSİUM. 16-19 May 1990. Atina. Abstract no: F26.
Koç Y, Güllü I, Akpek G, Akpolat T, Kansu E, Kiraz S, Batman F, Kansu T, Balkanci F, Akkaya S, Telatar H, Zileli T. Vascular involvement in Behçet’s disease. J Rheumatol 1992;19:402-10.
Lie JT. Vascular involvement in Behçet's disease: arterial and venous and vessels of all sizes. J Rheumatol 1992;19:341-3.
Melikoglu M, Ugurlu S, Tascilar K, et al. Large Vessel Involvement in Behçet’s Syndrome: A Retrospective Survey. Ann Rheum Dis 2008; 67(Suppl II):67.
Kansu E, Sivri B, Sahin G, et al. Endothelial cell dysfunction in Behçet’s disease. Editors: O’Duffy JD, Kökmen E. Behçet’s disease-basic and clinical aspects. New York: Marcel Dekker 1991;523-30.
Hamza M. Large artery involvement in Behçet’s disease. J Rheumatol 1987;14:554-9.
Gül A, Aslantas AB, Tekinay T, Koniçe M, Ozçelik T. Procoagulant mutations and venous thrombosis in Behçet's disease. Rheumatology (Oxford) 1999;38:1298-9.
Elbaz A, Poirer O, Canaple S, Chedru F, Cambien F, Amarenco P. The association between the Val34Leu polymorphism in the factor XIII gene and brain infarction. Blood 2000;95:586-91.
Leiba M, Seligsohn U, Sidi Y, Harats D, Sela BA, Griffin JH, Livneh A, Rosenberg N, Gelernter I, Gur H, Ehrenfeld M. Thrombophilic factors are not the leading cause of thrombosis in Behçet's disease. Ann Rheum Dis 2004;63:1445-9.
Rabinovich E, Shinar Y, Leiba M, Ehrenfeld M, Langevitz P, Livneh A. Common FMF alleles may predispose to development of Behcet's disease with increased risk for venous thrombosis. Scand J Rheumatol 2007;36:48-52.
Teter MS, Hochberg MC. Diagnostic criteria and epidemiology. In: Plotkin GR, Calabro JJ, O’Duffy JD, editors Behçet’s disease: A contemporary synopsis. New York: Futura publishing 1988;9-21.
Calamia KT, Wilson FC, Icen M, Crowson CS, Gabriel SE, Kremers HM. Epidemiology and clinical characteristics of Behçet's disease in the US: a populationbased study. Arthritis Rheum 2009;61:600-4.
Tursen U, Gurler A, Boyvat A. Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet's disease. Int J Dermatol. 2003;42:346-51.
Bang DS, Oh SH, Lee KH, Lee ES, Lee SN. Influence of sex on patients with Behçet's disease in Korea. J KoreanMed Sci 2003;18:231-5.
Dilşen N, Koniçe M, Aral O, Öcal L, İnanç M, Gül A. Risk factors for vital organ involvement in Behçet’s disease. In: Wecnsler B, Godeau P (eds) Behçet’s Disease, Elsevier, Amsterdam1993; pp:165-69.
Madanat W, Fayyad F, Verity D, Zureikat H. Influence ofsex on Behçet’s disease in Jordan. In Bang D, Lee ES, editors, Behçet’s disease: Proceedings of the 8th and 9th International Conference on Behçet’s disease. Seoul: Design Mecca Publishing 2001;90-3.
O’Neil TW, Rigby AS, McHugh S, Silman AJ, Barnes C. Regional differences in clinical manifestations of Behçet’s disease. In Godeau P, Wechsler B, eda. Behçet’s disease. Proceedings of the 6th International Conference on Behçet’s Disease: Amsterdam: Elsevier, 1993:159-63.
Zouboulis ChC, Djawari D, Kirch W, Ochsendorf F, Orfanos CE. Adamatiades Behçet’s disease in Germany. In: Godeau P, Wechsler B, eds. Behçet’s Disease in Germany. In: Godeau P, Wechsler B, eds. Behçet’s Disease. Amsterdam: Elsevier. Science Publishers 1993:193-196.
Tohmé A, Aoun N, El-Rassi B, Ghayad E. Vascular manifestations of Behçet's disease. Eighteen cases among 140 patients. Joint Bone Spine 2003;70:384-9.
Prokaeva T, Madanat W, Yermakova N, Alekberova Z. Sex dimorphism of Behçet’s disease. In: Godeau P, Wenchsler B, eds. Behçet’s disease. Amsterdam: Elsevier Sciense Publishers 1993:219-221.
Kötter I, Dürk H, Fieribeck G, et al. Behçet’s disease in 39 German and Mediterranean patients. In: Godeau P, Wechsler B, eds. Behçet’s Disease. Amsterdam: Elsevier Science Publishers, 1993:197-200.
Alkaabi JK, Pathare A. Pattern and outcome of vascular involvement of Omani patients with Behcet's disease. Rheumatol Int 2011;31:731-5.
Düzgün N, Ateş A, Aydintuğ OT, Demir O, Olmez U. Characteristics of vascular involvement in Behçet's disease. Scand J Rheumatol 2006;35:65-8.
Lê Thi Huong D, Wechsler B, Papo T, Piette JC, Bletry O, Vitoux JM, Kieffer E, Godeau P. Arterial lesions in Behçet's disease. A study in 25 patients. J Rheumatol 1995;22:2103-13.
Marcus AJ, Broekman MJ, Drosopoulos JH, Islam N, Alyonycheva TN, Safier LB, Hajjar KA, Posnett DN, Schoenborn MA, Schooley KA, Gayle RB, Maliszewski CR. The endothelial cell ecto-ADPase responsible for inhibition of platelet function is CD39. J Clin Invest 1997;99:1351-60.
al-Dalaan AN, al Balaa SR, el Ramahi K, al-Kawi Z, Bohlega S, Bahabri S, al Janadi MA. Behçet's disease in, Saudi Arabia. J Rheumatol 1994;21:658-61.
Ko GY, Byun JY, Choi BG, Cho SH. The vascular manifestations of Behçet's disease: angiographic and CT findings. Br J Radiol 2000;73:1270-4.
Schmitz-Huebner U, Knop J. Evidence for an endothelial cell dysfunction in association with Behçet's disease. Thromb Res 1984;34:277-85. 48.
Espinosa G, Font J, Tàssies D, Vidaller A, Deulofeu R, López-Soto A, Cervera R, Ordinas A, Ingelmo M, Reverter JC. Vascular involvement in Behçet's disease: relation with thrombophilic factors, coagulation activation, and thrombomodulin. Am J Med. 2002;112:37-43.
Akarsu M, Demirkan F, Ozsan GH, Onen F, Yüksel F, Ozkan S, Undar B. Increased levels of tissue factor pathway inhibitor may reflect disease activity and play a role in thrombotic tendency in Behçet's disease. Am J Hematol 2001;68:225-30.
Müftüoğlu AU, Yazici H, Yurdakul S, Tüzün Y,, Pazarli H, Güngen G, Deniz S.Behçet's disease. Relation of serum C-reactive protein and erythrocyte sedimentation rates to disease activity. Int J Dermatol 1986;25:235-9.
Lee YJ, Kang SW, Yang JI, Choi YM, Sheen D, Lee EB, Choi SW, Song YW. Coagulation parameters and plasma total homocysteine levels in Behcet's disease. Thromb Res 2002;106:19-24.
Sengül N, Demirer S, Yerdel MA, Terzioğlu G, Akin B, Gürler A, Tüzüner A. Comparison of coagulation parameters for healthy subjects and Behçet disease patients with and without vascular involvement. World J Surg 2000;24:1584-8.
Haznedaroglu IC, Ozcebe OI, Ozdemir O, Celik I, Dündar SV, Kirazli S. Impaired haemostatic kinetics and endothelial function in Behçet's disease. J Intern Med1996;240:181-7.
Hampton KK, Chamberlain MA, Menon DK, Davies JA. Coagulation and fibrinolytic activity in Behçet's disease. Thromb Haemost 1991;66:292-4.
Bertina RM, Koeleman BP, Koster T, Rosendaal FR, Dirven RJ, de Ronde H, van der Velden PA, Reitsma PH. Mutation in blood coagulation factor V associated with resistance to activated protein C. Nature 1994;369:64-7.
Fusegawa H, Ichikawa Y, Tanaka Y, Miyachi Y, Kawada T, Gondo K, Ikeda M, Shimizu H, Arimori S, Ando Y. Blood coagulation and fibrinolysis in patients withBehçet's disease. Rinsho Byori 1991;39:509-16.
Mader R, Ziv M, Adawi M, Mader R, Lavi I. Thrombophilic factors and their relation to thromboembolic and other clinical manifestations in Behçet's disease. J Rheumatol. 1999 ;26:2404-8.
Houman H, Lamloum M, Ben Ghorbel I, Khiari-Ben Salah I, Miled M. Vena cava thrombosis in Behçet's disease. Analysis of a series of 10 cases. Ann Med Interne (Paris) 1999;150:587-90.
Navarro S, Ricart JM, Medina P, Vayá A, Villa P, Todolí J, Estellés A, Micó ML, Aznar J, España F. Activated protein C levels in Behçet's disease and risk of venous thrombosis. Br J Haematol 2004 ;126:550-6.
Koşar A, Oztürk M, Haznedaroğlu IC, Karaaslan Y. Hemostatic parameters in Behçet's disease: a reappraisal. Rheumatol Int 2002;22:9-15.
Tokay S, Direskeneli H, Yurdakul S, Akoglu T. Anticardiolipin antibodies in Behçet's disease: a reassessment. Rheumatology (Oxford) 200;40:192-5.
al-Dalaan AN, al-Ballaa SR, al-Janadi MA, Bohlega S,Bahabri S. Association of anti-cardiolipin antibodies with vascular thrombosis and neurological manifestation of Behçets disease. Clin Rheumatol 1993;12:28-30.
Pereira RM, Gonçalves CR, Bueno C, Meirelles Ede S, Cossermelli W, de Oliveira RM. Anticardiolipin antibodies in Behçet's syndrome: a predictor of a more severe disease. Clin Rheumatol 1989;8:289-91.
Hull RG, Harris EN, Gharavi AE, Tincani A, Asherson RA, Valesini G, Denman AM, Froude G, Hughes GR. Anticardiolipin antibodies: occurrence in Behçet's syndrome. Ann Rheum Dis 1984 ;43(5):746-8.
Caramaschi P, Poli G, Bonora A, Volpe A, Tinazzi I, Pieropan S, Bambara LM, Biasi D. A study on thrombophilic factors in Italian Behcet's patients. Joint Bone Spine 2010;77:330-4.
Yaşar NŞ, Salgür F, Cansu DÜ, Kaşifoğlu T, Korkmaz C. Combined thrombophilic factors increase the risk of recurrent thrombotic events in Behcet's disease. Clin Rheumatol 2010;29:1367-72.
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