Impaired Prostacyclin Synthesis by Vessel Walls in Behçet's Disease

Kansu, Emin; Şahin, Gönül; Şahin, Fezan; Sıvrı, Bülent; Sayek, İskender; Batman, Figen

doi:10.1016/s0140-6736(86)90552-0

Cited by 31 publications

(13 citation statements)

References 2 publications

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“…Our results show that patients with pyrin mutations, while sharing major clinical features with their counterparts, had significantly more venous occlusive episodes (Tables 2 and 3 with an OR of 6.9). The exact pathogenesis of thrombosis in BD is poorly understood, but genetic defects and acquired changes affecting thrombosis, fibrinolysis, and endothelial function have been proposed as part of, or in addition to, vasculitis (20)(21)(22)(23)(24). Because vasculitis underlies BD and thrombosis is its direct or indirect consequence, our findings suggest that mutated pyrin may indeed be directly involved in the essence of the pathology that develops into BD.…”

Section: Discussionmentioning

confidence: 74%

Common FMF alleles may predispose to development of Behcet's disease with increased risk for venous thrombosis

Rabinovich¹,

Shinar²,

Leiba

et al. 2007

Scandinavian Journal of Rheumatology

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Section: Discussionmentioning

confidence: 74%

Common FMF alleles may predispose to development of Behcet's disease with increased risk for venous thrombosis

Rabinovich¹,

Shinar²,

Leiba

et al. 2007

Scandinavian Journal of Rheumatology

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“…Among these findings, the increase of HLA‐B51 in BD patients with thrombophlebitis compared to the patients without thrombophlebitis seems to be clinically important. High plasma endothelin activity, 13 defective production of prostacyclin by endothelial cells, 14 the presence of autoantibodies to endothelial cells, elevated levels of von Willebrand factor antigens, and circulating immune complexes are reported in BD patients with vascular involvement 15 . These factors lead to a thrombotic tendency and endothelial damage, which may constitute a new target for hyperfunctional neutrophils of HLA‐B51 positive BD patients; thus, HLA‐B51 may play an important role in the pathogenesis of thrombophlebitis in these patients.…”

Section: Discussionmentioning

confidence: 99%

Association of class I HLA antigens with the clinical manifestations of Turkish patients with Behçet's disease

Kaya

Türsen

Gürler³

et al. 2002

Clinical and Experimental Dermatology

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Genetic factors appear to be important in the pathogenesis of Behçet's disease. Although it is known to be strongly associated with HLA-B 51, the association of HLA class I antigens with specific clinical findings of the disease has not been studied extensively and the few studies are conflicting. The aim of this study was to investigate the association of HLA class I alleles with the manifestations of Behçet's disease in Turkish patients. Eighty-five patients with Behçet's disease were typed for HLA-A, B, and C antigens with the serologic, standard microlymphocytotoxicity technique. Possible associations of the HLA complex with clinical findings of Behçet's disease were examined. Statistically significant findings are as follows (P < 0.05): increased HLA-B 51 and decreased HLA-B35 frequency in patients with thrombophlebitis, increased HLA-A29 and decreased HLA-Bw6 frequency in patients with ocular involvement, decreased HLA-Cw2 frequency in patients with erythema nodosum, and decreased HLA-Cw 7 frequency in patients with genital ulceration. Of particular note, the results of this study suggest that the presence of HLA-B 51 and the absence of HLA-B35 can be regarded as laboratory risk factors of venous thrombosis in patients with Behçet's disease.

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“…52Thrombosis in the dural sinuses, superior and inferior vena cava and Budd-Chiari syndrome carry a poor prognosis 53. Reduced synthesis of prostacyclin by the endothelial cells,54defective fibrinolytic activity,55 and increased levels of endothelin-156 have all been reported in patients with Behçet's disease but these data do not correlate with clinical thrombosis.…”

Section: Clinical Presentationmentioning

confidence: 99%

Behçet’s disease

Kontogiannis

Powell

2000

Postgraduate Medical Journal

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Behçet's disease is a systemic vasculitis of unknown aetiology characteristically affecting venules. Onset is typically in young adults with recurrent oral and genital ulceration, uveitis, skin manifestations, arthritis, neurological involvement, and a tendency to thrombosis. It has a worldwide distribution but is prevalent in Japan, the Middle East, and some Mediterranean countries. International diagnostic criteria have been proposed, however diagnosis can be problematical, particularly if the typical ulcers are not obvious at presentation. Treatment is challenging, must be tailored to the pattern of organ involvement for each patient and often requires combination therapies. (Postgrad Med J 2000;76:629-637)

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Impaired Prostacyclin Synthesis by Vessel Walls in Behçet's Disease

Cited by 31 publications

References 2 publications

Common FMF alleles may predispose to development of Behcet's disease with increased risk for venous thrombosis

Common FMF alleles may predispose to development of Behcet's disease with increased risk for venous thrombosis

Association of class I HLA antigens with the clinical manifestations of Turkish patients with Behçet's disease

Behçet’s disease

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