endothelin-1 and Behcet-Syndrome

Plasma concentrations of endothelin-1 (ET-1), a vasoconstrictor peptide released from vascular endothelial and synovial cells, were measured by radioimmunoassay in 27 patients with Behçet's disease (BD) and 18 healthy controls. The plasma ET-1 concentrations were found to be significantly increased in patients with BD (121.15 +/- 11.6 fmol/dl) compared with the healthy subjects (89.37 +/- 8.03 fmol/dl p < 0.05). The elevation of ET-1 did not correlate with the disease duration, gender, ocular or articular involvement. Whether the elevated ET-1 level is a direct result of its increased synthesis from injured vascular endothelial cells or whether its presence in high concentrations may be responsible, together with other factors for both vascular and articular complications in patients with BD is uncertain.

Topics: Adult; Behcet Syndrome; Biomarkers; Endothelin-1; Female; Humans; Male; Radioimmunoassay; Sensitivity and Specificity

Pulmonary aneurysms and thrombosis constitute a significant cause of morbidity and mortality in Behçet's disease (BD). Various factors have been studied to explore the pathogenesis of vascular involvement in BD. As endothelin (ET) is known for its potent vasoconstrictor and proinflammatory properties, we supposed that it is involved during the inflammatory process of BD pulmonary vasculitis.. To investigate the role of ET in BD, ET-1 concentrations were measured in bronchoalveolar lavage fluid (BALF) of 18 nonsmoking BD patients with pulmonary manifestations and 12 control subjects. Immunoreactivity of ET-1 was also evaluated in alveolar macrophages (AMs) cytoplasm.. ET-1 levels in BD-BALF were significantly higher than those of controls. ET-1 levels were correlated with the number of alveolar macrophages, but not with BAL-CD4/CD8 ratio. ET-1-immunoreactivity was found mainly in AM of BD-BAL.. Increased ET-1 production from AM is associated with pulmonary BD manifestations.

Topics: Adult; Behcet Syndrome; Bronchoalveolar Lavage Fluid; CD4 Antigens; CD8 Antigens; Endothelin-1; Female; Humans; Immunoassay; Immunohistochemistry; Macrophages, Alveolar; Male; Middle Aged

Behçet's disease (BD) is a systemic inflammatory vasculitis of young adults with unknown aetiology, characterised by endothelial dysfunction and occlusion in both deep venous and retinal circulation. Ocular involvement occurs in 70% of cases and is characterised by periphlebitis, periarteritis, vascular occlusion, and thrombosis leading to blindness despite vigorous treatment. Endothelin-1 (ET-1) is a vasoconstricting peptide while nitric oxide (NO) is a relaxing molecule and both are released by endothelium for blood flow regulation. Homocysteinaemia is a newly defined term connected to the increased risk of atherothrombotic and atherosclerotic systemic and retinal vascular occlusive diseases, and its role in the course of BD has not been previously described. The authors aimed to detect serum total homocysteine (tHcy), ET-1, and NO in BD and to assess if tHcy, ET-1, and NO are associated with ocular BD or disease activity.. 43 consecutive patients with ocular (n = 27) or non-ocular (n = 16) BD (36.95 (SD 9.80) years, 22 male, 21 female) satisfying international criteria, and 25 age and sex matched healthy control subjects (37.88 (8.73) years, 13 male, 12 female) without a history of systemic or retinal venous thrombosis were included in this study. Patients were examined by two ophthalmologists with an interest in BD. Serum tHcy, ET-1, and NO concentrations were measured in both groups. Hyperhomocysteinaemia was defined as a tHcy level above the 95th percentile in the control group. Patients were divided into active and inactive period by acute phase reactants including alpha(1) antitrypsin, alpha(2) macroglobulin, erythrocyte sedimentation rate, and neutrophil count.. The overall mean serum tHcy, ET-1, and NO levels were significantly higher in patients with BD than in control subjects (tHcy = 15.83 (4.44) v 7.96 (2.66) ng/ml, p <0.001; ET-1 = 17.47 (4.33) v 5.74 (2.34) micromol/ml, p <0.001; NO = 37.60 (10.31) v 27.08 (7.76) micromol/l, p <0.001). Serum tHcy, ET-1, and NO levels were significantly higher in active patients than in inactive patients and control subjects. In addition, among patients with ocular BD, the mean tHcy levels were significantly increased and correlated with ET-1 and NO levels when compared with non-ocular disease and control subjects. All acute phase reactant levels were significantly higher in active period than in inactive stage and controls.. Elevated tHcy may be responsible for the endothelial damage in BD and may be an additional risk factor for the development of retinal vascular occlusive disease, contributing to the poor visual outcome in these patients. Assessment of tHcy may be important in the investigation and management of patients with BD, especially with ocular disease.

Topics: Acute-Phase Proteins; Adolescent; Adult; Behcet Syndrome; Biomarkers; Blood Sedimentation; Endothelin-1; Female; Homocysteine; Humans; Hyperhomocysteinemia; Leukocyte Count; Male; Middle Aged; Neutrophils; Nitric Oxide; Retinal Diseases

Topics: Adult; Behcet Syndrome; Case-Control Studies; Endothelin-1; Female; Humans; Male; Middle Aged; Vasoconstriction

Topics: Behcet Syndrome; Blood Sedimentation; C-Reactive Protein; Endothelin-1; Humans