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Deep Vein Thrombosis in Behcet's Disease

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Deep Vein Thrombosis in Behcet's Disease BRIEF PAPER Clinical and Experimental Rheumatology 2001; 19 (Suppl. 24): S48-S50. Deep vein thrombosis ABSTRACT stitute the most frequent vascular mani- Objective festation seen in 6.2 to 33 % cases of in Behcet’s disease We aimed to describe the epidemiologi - BD (1, 2). We carried out this study to cal and clinical aspects of deep vein d e t e rmine the fre q u e n cy, the cl i n i c a l M.H. Houman1 thrombosis (DVT) in Behçet’s disease characteristics and course of deep vein 1 (BD) and to determine the patients at thrombosis (DVT) in BD patients and I. Ben Ghorbel high risk for this complication. to define a subgroup of patients at high I. Khiari Ben Salah1 Methods risk for this complication. M. Lamloum1 Among 113 patients with BD according 2 to the international criteria for classifi - Patients and methods M. Ben Ahmed cation of BD, those with DVT were ret - The medical records of one hundred M. Miled1 rospectively studied.The diagnosis of and thirteen patients with BD were re- DVT was made in all cases using con - viewed in order to investigate the pa- 1Department of Internal Medicine. La ventional venous angiography, venous tient’s medical history, the clinical ma- Rabta Hospital, 2Department of Immuno- ultrasonography and/or thoracic or ab - nifestations and outcome of the disease logy, Institute Pasteur. Tunis, Tunisia. dominal computed tomograp hy. Pa - as well as the treatment prescribed.The Houman M Habib, MD; Ben Ghorbel tients were divided in two subgroups diagnosis of BD was made based on the Imed, MD; Khiari Ben Salah Imen; a c c o rding to the occurrence of DV T criteria established by the international Lamloum Mounir, MD; Ben Ahmed other than cereb ral thromboses. Th e study group for BD (3). Patients were Malika, MD; Miled Mohamed, MD. medical records of these patients were divided in two subgroups according to Please address correspondence to: reviewed in order to investigate their the occurrence of DVT other than cere- Pr. M. Habib Houman, Department of Internal Medicine, Hospital La Rabta past medical history and evaluate their bral thrombosis. The diagnosis of DVT 1007 Tunis, Tunisia. response to the treatment prescribed. was made using venous ultrasonogra- E-mail: [email protected] Clinical and genetic factors (HLA B51 phy in all cases, with abdominal com- Received on November 30, 2000; and MICA 6) that might contribute to puted tomography in 8 cases for inferi- accepted in revised form on April 6, 2001. DVT were analysed by comparing pa - or vena cava thrombosis (IVCT) and © Copyright CLINICAL AND tients with and without DVT. Results of t h o racic computed tomograp hy in 4 EXPERIMENTAL RHEUMATOLOGY 2001. our series were compared to those of cases for superior vena cava thrombo- other series in the literature. Statistical sis (SVCT); conventional venous an- Key words: Behçet’s disease, deep analysis was by Chi square with neces - giography was performed in one case. vein thrombosis, HLA linkage, MIC sary correction and Fischer tests. Protein S, protein C and anti-thrombin genes. Results III levels were determined in all pa- Forty-four patients (38.9%) had deep tients. The anticardiolipin (aCL) and vein thrombosis of various systems with antiß2 Gly c o p rotein1 antibodies 81 localisations. Th e re we re 40 men (ß2GP1) were measured in 24 patients and four women (mean age 28.1 years; by ELISA using IgG isotype. HLA- range 17-60). DVT appeared after the B51 allele was determined in 38 pa- onset of disease with a mean delay of tients using a complement-dep e n d e n t 3.8 ye a rs. In 6 cases, DVT reve a l e d m i c ro ly m p h o cyte toxicity assay; fi f- BD.When we evaluated the risk of DVT teen of these patients had DVT. Triplet coexistence with other clinical findings rep e at poly m o rphism of MICA wa s and genetic fa c t o rs (HLA B51 and analysed on a denaturating polyacryna- MICA 6), we found a significant posi - mide gel and alleles were visualised by tive correlation with sex, and positive autoradiograghy in 34 patients, 11 of pathergy test. whom had DVT. Results in both sub- Conclusion groups were compared by Chi-square In our series, occurrence of DVT was with necessary correction and Fischer significantly associated with male gen - tests. der and positive pathergy test. Results Introduction Of 113 patients with BD, 49 (43.3%) Behçet’s disease (BD) is a multisystem had vascular invo l vement. A m o n g disorder characterised mainly by recur- them 44 (38.9%) patients had DVT, 7 rent oral and genital ulcers and ocular arterial aneurysms and 6 arterial throm- involvement. Neurologic and vascular boses. Seven patients presented both involvement are not rare and may be venous and arterial involvements. The life-threatening.Vein thromboses con- group of patients with DVT consisted S-48 Deep vein thrombosis in Behçet’s disease / M.H. Houman et al. BRIEF PAPER of 40 men and four women whereas the Table I. Clinical and genetic features of BD patients with and without DVT. group of the remaining 64 pat i e n t s without DVT was composed of 37 men Behçet’s disease Behçet’s disease with DVT without DVT P and 27 women. Male pre d o m i n a n c e n = 44 n = 64 was significantly higher in the DVT pa- tient group (p = 0.0004). Mean age of Male 40 (90.9%) 37 (57.8%) 0.0004 patients at the moment of diagnosis of Age (years) 28.4 32 0.562 BD was ro u g h ly similar for pat i e n t s Buccal aphtosis 43 (97.7%) 64 (100%) 0.407 with (28.1 years) and without DVT (32 Genital aphtosis 33 (75%) 48 (75%) 0.821 years). The average delay to diagnosis Pseudofolliculitis 25 (56.8%) 30 (46%) 0.412 of DVT from the date of BD diagnosis Pathergy Test 34 (77.2%) 37 (57%) 0.036 was 3.1 years (range 0-18 years). DVT Erythema nodosum 20 (45.4%) 34 (53%) 0.556 revealed BD in 6 cases. Eighty-one lo- Ocular involvement 14 (31.8%) 30 (46%) 0.112 cations of DVT were detected. Forty- Articular involvement 26 (59%) 44 (68%) 0.407 three patients showed more than one Neurological 8 (18.1%) 6 (9%) 0.231 location. Twelve patients had a vena HLA B51 11 (25%) 13 (20%) 0.480 c ava thrombosis (VCT) among them MICA 6 8 (18%) 20 (31%) 0.287 only one had both superior and inferior V C T. Hep at i c ve n o u s t h ro m b o s i s Budd-Chiari syndrome. Of 20 patients (62% of cases) and this result is also in (Budd-Chiari syndrome) was seen in 5 with DVT treated with corticosteroids, agreement with those rep o rted by p atients. Clinical fe at u res of BD in 5 showed re c u rrence of thro m b o s i s , Wechsler (8). The second most com- p atients with and without DVT are while 4 of the remaining 24 patients mon localization of DVT was the vena summarised and compared in Table I. had this complication (p = 0.72). cava, observed in 10 patients. VCT was Pat h e rgy test was signifi c a n t ly more reported in 0.2 to 10%, more frequently f re q u e n t ly positive in patients with Discussion in West Mediterranean and European DVT (p = 0.030). Protein C, protein S Although vascular lesions are not in- patients (9). In our study, 6 patients had and anti-thrombin III levels were nor- cluded in the major diagnostic criteria hepatic venous thrombosis which is a mal in all patients. Seven of 24 patients of BD, our results and other reported very rare complication of BD reported were positive for IgG aCL with no dif- investigations indicate that 1/4 to 1/2 of in 0.3 to 2.8 % of cases (7). fe rence between patients with and patients are likely to develop this com- In this study, pathergy positivity was without DVT, and no patient was posi- plication (2, 4). Venous thrombosis ap- the only statistically significant clinical tive for aß2GP1. Eleven patients with p e a red to be the major vascular in- feature which is more frequently ob- DVT and thirteen patients without vo l vement rep o rted in 7 to 33% of served in BD patients with DVT com- DVT were HLA B51 positive, the dif- cases with BD, and representing 85 to pared with those without DVT. A high- ference was not statistically significant 93% of vasculo-Behçet. It is signifi- er prevalence of positive pathergy test (p = 0.480). Eight patients with DVT c a n t ly more fre q u e n t ly observed in and erythema nodosum in vasculopathy and twenty patients without DVT were A rab and European populations (19- BD had also been previously reported MICA 6 positive, the difference was 34%) (5) than in Asian non-Arab popu- by Koç (4) and Muftüoglu (2) fro m not statistically significant either (p = lations (7-12.5%) (6). Turkey. 0.287). Our study confirms the male predomi- It is well known that HLA B51 is the All patients were treated with anticoag- nance reported by all previous studies most important genetic factor associat- ulant agents and colchicine (1mg/day).
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