Factor VIII Related Antigen (von Willebrand's Factor) In Kawasaki Disease

Irazuzta, José; Elbl, Francisco; Rees, Allan R.

doi:10.1177/000992289002900613

Cited by 11 publications

(6 citation statements)

References 5 publications

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“…( + 0 4 to +23,0 04) +13 ng/ml* ( + 2 to +24,0-03) -2 ng/ml (-47 to +43.0 94) +13 ng/ml (-51 to +77.0-67) -1% (-9 to +7,0-75) -3% (-8 to +3.031) 'P < 0-05 compared wilh samples separated at 1 h and defrosted once. [28,29], and we have confirmed this. However, levels were no different in K.D from those in febrile controls.…”

Section: Discussionsupporting

confidence: 82%

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Soluble cell adhesion molecules and von Willebrand factor in children with Kawasaki disease

Nash¹,

Shah²,

Dillon³

1995

Clinical and Experimental Immunology

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SUMMARYFifty-nine children with acute Kawasaki disease (KD), a childhood vasculitis.. were compared with 35 children with fever due to infection and 48 healthy childreti. Levels of soluble E-selectin (sEselectin), soluble intercellular adhesion molecule-1 (s!CAM-I), and soluble vascular cell adhesion molecule-1 (sVCAM-I) in ihe healthy children were double Ihose found in adults. Al! Ihree soluble cell adhesion molecules and von Willebrand factor (vWF) were higher in the children wilh KD than in the healthy children, but only sE-selectin, a marker for activated endothelial cells, and slCAM-1 were higher than in the febrile children. The high levels of vWF in KD appear to reflect the prominent acute-phase reaction. This information can help us to understand further the complex interactions between cytokines. circulating inflammatory cells and the vascular endothelium. and may lead to aew tlwrapeutic avenues in KD and other inflammatory diseases and vasculitides.

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Section: Discussionsupporting

confidence: 82%

“…VWF has been previously found to be elevated in .small groups of children with KD compared with afebrile controls 'P < 0-05 compared wilh samples separated at 1 h and defrosted once. [28,29], and we have confirmed this. However, levels were no different in K.D from those in febrile controls.…”

Section: Discussionsupporting

confidence: 82%

Soluble cell adhesion molecules and von Willebrand factor in children with Kawasaki disease

Nash¹,

Shah²,

Dillon³

1995

Clinical and Experimental Immunology

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“…37 These observations suggest that mediator secretion by activated T cells and macrophages could promote vascular injury in Kawasaki disease. A sizeable literature has reported on the immune response in the acute phase of Kawasaki disease, including elevation of TNF-␣, 38 44 and cysteinyl leukotrienes, 45 whereas the serum concentration of transforming growth factor-␤1 decreases in acute Kawasaki disease. 46 In addition, anti-endothelial cell antibodies present in sera from Kawasaki disease patients may contribute to vascular injury in Kawasaki disease.…”

Section: Etiology and Pathogenesismentioning

confidence: 99%

“…A sizeable literature has reported on the immune response in the acute phase of Kawasaki disease, including elevation of TNF-␣, 38 various interleukins (e.g. IL-1␤, 38 IL-4, 39 IL-6 40 and IL-10 41 ), circulating soluble sel-Vascular Medicine 1999; 4: 187-202 ectins, 42,43 Factor VIII-related antigen (von Willebrand's Factor), 44 and cysteinyl leukotrienes, 45 whereas the serum concentration of transforming growth factor-␤1 decreases in acute Kawasaki disease. 46 In addition, anti-endothelial cell antibodies present in sera from Kawasaki disease patients may contribute to vascular injury in Kawasaki disease.…”

Section: Etiology and Pathogenesismentioning

confidence: 99%

Kawasaki disease

Burns

1999

Vasc Med

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Kawasaki disease is a leading cause of acquired heart disease in children in the USA. An acute vasculitis of unknown etiology, it occurs predominantly in infancy and early childhood, and more rarely in teenagers. Coronary artery aneurysms or ectasia develop in approximately 15-25% of children with the disease. Treatment with intravenous gamma globulin, 2 g per kg, in the acute phase reduces this risk three-to fivefold. Angiographic resolution occurs in approximately one-half of aneurysmal arterial segments, but these show persistent histologic and functional abnormalities. The remainder continue to be aneurysmal, often with development of progressive stenosis or occlusion. The worst prognosis occurs in children with so-called 'giant aneurysms', i.e. those with a maximum diameter greater than 8 mm, because thrombosis is promoted both by sluggish blood flow within the massively dilated vascular space and by the frequent development of stenotic lesions. Serial stress tests with myocardial imaging are mandatory in the management of patients with Kawasaki disease and significant coronary artery disease to determine the need for coronary angiography and transcatheter interventions or coronary bypass surgery. Continued long-term surveillance in patients with and without detected coronary abnormalities is necessary to determine the natural history of Kawasaki disease.

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“…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: 98%

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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Factor VIII Related Antigen (von Willebrand's Factor) In Kawasaki Disease

Cited by 11 publications

References 5 publications

Soluble cell adhesion molecules and von Willebrand factor in children with Kawasaki disease

Soluble cell adhesion molecules and von Willebrand factor in children with Kawasaki disease

Kawasaki disease

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

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