Evidence for an endothelial cell dysfunction in association with Behçet's disease

Schmitz-Huebner, U.; Knop, J.

doi:10.1016/0049-3848(84)90384-0

Cited by 77 publications

(57 citation statements)

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“…1 It has been shown that the common histological lesion of all clinical manifestations appears to be vasculitis. 4,5 Many studies concerning the vascular involvement and the thrombotic Also, the finding of decreased plasminogen fibrinolytic activity in patients with active BD presents a possible disturbance in the blood coagulation mechanism in BD patients. 6 Notably, the issue of CV involvement in BD, which was thought to be a relatively rare manifestation of the disease, has attracted much attention of cardiologists over the past few decades, owning to the widespread use of highly informative diagnostic techniques, such as echocardiography.…”

Section: Discussionmentioning

confidence: 99%

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Cardiovascular Involvement in Behçet Disease: Clinical Implications

et al. 2015

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Beh?et disease (BD) is a multisystem disorder, with vasculitis as its underlying pathological process, in contrast to the classic triad of recurrent oral and genital ulcerations, with uveitis. Vascular involvement in BD includes venous thrombosis, arterial occlusion, and pulmonary artery and aortic aneurysm formation. Cardiac involvement is rare and often obscure. It includes intracardiac thrombi formation, and is associated with a poor prognosis. Our objectives are to describe two cases with BD, complicated with vascular and cardiac involvement, and to raise awareness of these rare complications, the needed routine surveillance, and thus to prevent inappropriate interventions, serious outcomes, and mortality. We present two male patients from the Mediterranean Basin with BD. The first was diagnosed early as a BD patient. The second was diagnosed at the time of cardiovascular (CV) involvement. We recommend that patients who are diagnosed, or even suspected of suffering from BD, especially in endemic areas along the Silk Route pathway, should be followed up routinely for CV involvement, even if rare, obscure, or with a bizarre presentation.

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Section: Discussionmentioning

confidence: 99%

“…[1][2][3][4] Vasculitis is the underlying pathological process, in contrast to the classic triad of recurrent urogenital ulcerations, with uveitis. 5,6 Vascular involvement in BD includes venous thrombosis, arterial occlusion, pulmonary artery, and aortic aneurysm formation. [7][8][9][10][11][12] Cardiac involvement is rare, often obscure.…”

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confidence: 99%

Cardiovascular Involvement in Behçet Disease: Clinical Implications

et al. 2015

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“…The pathogenic mechanism underlying thrombotic propensity in patients with BD is not completely understood. It is however reported that endothelial cell ischemia or disruption leads to enhancement of platelet aggregation (9). So far, none of the thrombophilic factors were shown to be associated with the thrombotic tendency observed in BD.…”

Section: Casementioning

confidence: 99%

Intracardiac thrombus in Behçet’s disease

2016

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Behçet’s disease (BD) is a multisystem inflammatory disorder. Intracardiac thrombus (ICT) formation is an uncommon but important complication of BD. Of the cases of Behçet’s disease, we selected those with ICT. All patients fulfilled the diagnostic criteria of the International Study Group of Behçet’s disease. The ICT in each case was confirmed by ultrasonography, computed tomography and MRI. Clinical features and laboratory parameters were determined. Among our 518 patients with BD, 8 were diagnosed as having intracardiac thrombus (ICT). All were male; the mean age at the time of the ICT diagnosis was 30.8 years. The main presenting symptoms were hemoptysis, chest pain, and dyspnea. It was associated with pulmonary artery aneurysm and vena cava thrombosis in 3 cases each, pulmonary embolism, and lower limbs deep venous thrombosis in 1 case each. The coexistence of other cardiac complications was as follows: pericarditis in 2 cases, myocarditis, endomyocardial fibrosis, and coronary arteritis with consequent myocardial infarction in one case each. In all cases, echocardiography was sufficient to reach the diagnosis. Chest computed tomography performed in all cases led to the diagnosis of associated pulmonary vasculo-Behçet lesions in 4 cases. All patients received colchicine, anticoagulation, and corticosteroids. Seven patients were on immunosuppressant agents (2 patients received azathioprine and 5 cyclophosphamide). Clinical remission with ICT resolution was observed in 5 cases. Combined immunosuppressive therapy with prednisone and cyclophosphamide might be needed to treat ICT due to BD.

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“…122 These patients have been found to have a depressed blood fibrinolysisl23.124 and to release low amounts of plasminogen activator following vasogenic stimuli as injection of DDAVP, a vasopressin analogue. 123 In 12 cases of thrombotic thrombocyto penic purpura plasminogen activator activity was found to be unmeasurably low but the levels of t-PA antigen were normal.I25 The finding could be explained by the presence in all 12 cases examined of an inhibitor directed to t-PA and u-PA. Low levels of protein C antigen presumably had a contributory action to the decreased activity.…”

Section: Miscellaneous Conditionsmentioning

confidence: 99%

The role of fibrinolytic factors in ischaemia

Pandolfi

al-Rushood

1991

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SummaryThe fibrinolytic system is an enzymatic cascade system whose activation leads to for mation of a trypsin-like serine protease, plasmin, which splits insoluble fibrin into soluble degradation products. It is believed that the main function of fibrinolysis is defence against thrombotic occlusion of vessels and dissolution of thrombi once they are formed (thrombolysis).The authors review the recent literature providing evidence that fibrinolysis plays a role in the pathogenesis of vascular occlusions. From earlier studies based on global assay methods it is known that fibrinolysis is depressed in patients with vascular occlusions. Selective assay methods show that almost invariably the fibrinolytic activity of these patients is depressed either following increased levels of fibrinolytic inhibitors (mainly plasminogen activator inhibitor I or PAl-I) and/or decreased· levels of a plasminogen activator (tissue plasminogen activator or t-PA). In a few cases the molecule of plasminogen shows a conformational abnormality making it less susceptible to conversion to plasmin.In the last decade numerous studies have been published showing a connection between a depressed fibrinolysis and venous thrombosis. In patients with coronary artery occlusion fibrinolysis is depressed mainly because of increased levels of PAl-I.Hypertriglyceridaemia seems to aggravate the defective fibrinolysis. There is also evidence of a decreased fibrinolysis in patients with peripheral ischaemic diseases.A depressed fibrinolysis has also been documented in states predisposing to vas cular occlusions. Thus Iwo levels of t-PA/increased levels of PAI-I have been found in obesity, diabetes mellitus, postoperative states, SLE, malignancies, and miscellan eous diseases often complicated with thrombosis such as Beh�et's syndrome. In preg nancy fibrinolysis is depressed because of the presence in blood of PIA-2, an inhibitor of plasminogen activators secreted by the placenta. The Fibrinolytic SystemThe fibrinolytic system is an enzymatic cas cade system whose activation leads to forma tion of a trypsin-like serine protease, plasmin, which is capable of degrading fibrin as well as fibrinogen, factor V and VIII. It is believed that the main function of fibrinolysis is defence against thrombotic occlusion of vessels, dissolution of thrombi once they are formed (thrombolysis) and resolution of clots and fibrinous exudates occurring in various parts of the body. Activators of fibrinolysisA simplified scheme of the fibrinolytic system in man is shown in Figure 1.

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Evidence for an endothelial cell dysfunction in association with Behçet's disease

Cited by 77 publications

References 11 publications

Cardiovascular Involvement in Behçet Disease: Clinical Implications

Cardiovascular Involvement in Behçet Disease: Clinical Implications

Intracardiac thrombus in Behçet’s disease

The role of fibrinolytic factors in ischaemia

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