Von Willebrand factor antigen levels in Behçet disease

Beyan, Eśin; Sadikoglu, Begüm; Ertuğrul, Esin; Beyan, Cenǵiz

doi:10.1002/ajh.20317

Cited by 8 publications

(5 citation statements)

References 7 publications

(8 reference statements)

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“…[44][45][46] Moreover, high levels of serum concentration of von Willebrand factor (vWF, an endothelial product that is shown in yellow in Figure 4), plasminogen activator inhibitor-1, and thrombomoduline were found in patients with BD. 47,48 Along this line, Demirer et al, 46 Ozoran et al, 47 and Beyan et al 49 showed that the level of vWF was significantly higher in patients with BD, supporting endothelial destruction because of vasculitis related with BD. 50 Factor V Leiden (F5) mutation was also reported as associated with thrombosis in BD.…”

Section: Focal Adhesionmentioning

confidence: 76%

Identification of possible pathogenic pathways in Behçet’s disease using genome-wide association study data from two different populations

et al. 2014

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Behc¸et's disease (BD) is a multi-system inflammatory disorder of unknown etiology. Two recent genome-wide association studies (GWASs) of BD confirmed a strong association with the MHC class I region and identified two non-HLA common genetic variations. In complex diseases, multiple factors may target different sets of genes in the same pathway and thus may cause the same disease phenotype. We therefore hypothesized that identification of disease-associated pathways is critical to elucidate mechanisms underlying BD, and those pathways may be conserved within and across populations. To identify the disease-associated pathways, we developed a novel methodology that combines nominally significant evidence of genetic association with current knowledge of biochemical pathways, protein-protein interaction networks, and functional information of selected SNPs. Using this methodology, we searched for the disease-related pathways in two BD GWASs in Turkish and Japanese case-control groups. We found that 6 of the top 10 identified pathways in both populations were overlapping, even though there were few significantly conserved SNPs/genes within and between populations. The probability of random occurrence of such an event was 2.24E À39. These shared pathways were focal adhesion, MAPK signaling, TGF-b signaling, ECM-receptor interaction, complement and coagulation cascades, and proteasome pathways. Even though each individual has a unique combination of factors involved in their disease development, the targeted pathways are expected to be mostly the same. Hence, the identification of shared pathways between the Turkish and the Japanese patients using GWAS data may help further elucidate the inflammatory mechanisms in BD pathogenesis.

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Section: Focal Adhesionmentioning

confidence: 76%

Identification of possible pathogenic pathways in Behçet’s disease using genome-wide association study data from two different populations

et al. 2014

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“…Previous reports found contradicting results regarding the correlation between vWF plasma level and active vasculitis (11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24). Most of the studies, however, were small retrospective case series, with varying non-validated de nitions for active vasculitis and remission (16,20,21,22).…”

Section: Discussionmentioning

confidence: 99%

“…Increased serum levels of vWF antigen in different systemic vasculitides have been demonstrated in various studies, including in Kawasaki disease (11)(12)(13)(14), childhood primary CNS vasculitis (6), polymyalgia rheumatica (15), giant cell arteritis (15)(16)(17), granulomatosis with polyangiitis (18, 19), microscopic polyangiitis (19), Henoch-Schönlein purpura (20)(21)(22), Takayasu arteritis (23) and Behçet's disease (24). Most of these studies, however, are small retrospective case series that did not use validated outcome measures (16,(20)(21)(22)(23), and vWF Ag serum levels were not corrected to blood groups.…”

Section: Introductionmentioning

confidence: 99%

Von Willebrand factor: a possible biomarker for disease activity in vasculitis

Keret,

Mazareeb,

Snir

et al. 2024

Scandinavian Journal of Rheumatology

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Background:In ammation markers commonly used to evaluate disease activity in vasculitis, C-reactive protein (CRP) and sedimentation rate, are often normal despite active disease. Von -Willebrand factor (vWF), is synthesized primarily in endothelial cells and secreted in response to vascular damage. Sporadic reports suggested increased vWF levels in vasculitis. Aim:To evaluate vWF serum concentration in patients with vasculitis as a marker of disease activity. Methods:Adult patients with systemic vasculitis were prospectively enrolled. Disease activity was recorded using the Birmingham Vasculitis Activity Score (BVAS) v3. Blood group adjusted vWF antigen serum level was evaluated at diagnosis and when available after treatment. Results:Twenty-ve patients with systemic vasculitis were compared to 15 healthy controls. The mean age of patients was 56 ± 17 years and 56% were women. 40% had ANCA-associated vasculitis; 20% had giant cell arteritis; 16% had polyarteritis nodosa; 8% had Takayasu arteritis and the rest had other vasculitides.The mean disease duration was 3.4 ± 4.8 years. Mean vWF was higher in patients with active vasculitis compared to patients in remission or low disease activity state and healthy controls-212%±81, 159%±80, and 106%±26, respectively. vWF levels directly correlated with BVAS. In 13 patients with active vasculitis who reached remission or LDA after treatment, vWF level at follow-up decreased signi cantly. In 3/5 patients who were treated with IL-6 inhibitors, vWF was elevated despite normal CRP levels, while clinically vasculitis was active. Conclusion:vWF Ag serum level is increased in active vasculitis and is suggested as a biomarker for disease activity. Key points * Traditional (CRP / ESR) in ammatory markers may fail to re ect active in ammation in patients with vasculitis. * Von -Willebrand factor is directly related to endothelial damage. * Von -Willebrand factor is elevated in patients with active vasculitis, and is correlated with disease activity as measured by BVAS score.* Von-Willebrand factor levels may serve as an additional biomarker for monitoring disease activity and treatment response.

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“…105 More recently, significantly higher vWF: Ag levels were reported in BD patients compared to a control group. 106 There was no statistically significant difference between vWF: Ag levels in patients with and those without organ involvement. A significant linear correlation between high vWF: Ag and serum ferritin levels was observed, suggesting that vWF: Ag was related to disease activity.…”

Section: Coagulation Factorsmentioning

confidence: 94%

“…A significant linear correlation between high vWF: Ag and serum ferritin levels was observed, suggesting that vWF: Ag was related to disease activity. 106 BD with deep vein thrombosis and the factor V Leiden mutation has been reported. 107 However, others authors have not found any association between three thrombogenic mutations (factor V gene G169IA, methylenetetrahydrofolate reductase gene C677T, and prothrombin G →A 20210 mutation) and BD patients with thrombosis.…”

Section: Coagulation Factorsmentioning

confidence: 97%

Behçet’s disease: an update on pathogenesis, diagnosis and management of vascular involvement

Kaklamanis

Calamia

2010

Rheumatol Rep

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The objective of this review is to summarize reports of the prevalence, clinical presentation, diagnostic methodology and treatment of vasculitic manifestations of Behçet’s disease (BD). We performed a literature search on vasculitis in BD. Articles were selected which provided insight into the pathogenesis and clinical aspects of vasculitis. Vasculitis underlies many of the clinical features of BD. Small vessel vasculitis is often found in the pathology of the mucocutaneous manifestations of BD. Large vessel vasculitis has been reported in 15-40% of BD patients. Ultrasound, angiography and tomography are applied to confirm the diagnosis when venous involvement is suspected. Endothelial dysfunction plays a role in the pathogenesis of disease. Peripheral arterial involvement in BD occurs in the form of arterial occlusion or aneurysms. Pulmonary arterial involvement is often life-threatening. The cause of cardiac vascular involvement requires an aggressive diagnostic approach. Corticosteroids and immunosuppressive agents have been used successfully in the early stage of large vessel disease and should be used as an adjunct to surgery. An increasing amount of data is available regarding the role of anti-tumor necrosis factor (TNF) agents for the treatment of BD. Anticoagulant therapy may be hazardous in patients with aneurysmal dilatation of the pulmonary vascular tree and is not effective in the treatment of venous thrombosis. Inflammation of small and large vessels is very frequent in BD. Both arteries and veins may be involved. Early recognition and appropriate management of large vessel vasculitis in BD is essential to reduce associated morbidity and mortality

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Von Willebrand factor antigen levels in Behçet disease

Cited by 8 publications

References 7 publications

Identification of possible pathogenic pathways in Behçet’s disease using genome-wide association study data from two different populations

Identification of possible pathogenic pathways in Behçet’s disease using genome-wide association study data from two different populations

Von Willebrand factor: a possible biomarker for disease activity in vasculitis

Behçet’s disease: an update on pathogenesis, diagnosis and management of vascular involvement

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