Aim: Behçet’s disease (BD) is a chronic, inflammatory disease effecting multiple organs. Vascular involvement is a major cause of morbidity and mortality in BD patients. Though clinically well-defined, there is limited information related to disease pathogenesis and vascular incidences in this patient group. The aim of the current study is to investigate the unique metabolic signatures of BD patients with vascular involvement (VBD). Methods: Metabolomic profiling was performed on serum samples of 48 BD patients (18 with vascular involvement) and 40 healthy controls using gas chromatography-mass spectrometry (GC-MS) based untargeted metabolomics analysis. Multivariate and univariate statistical analyses were performed to find altered metabolites and pathways. Results: Untargeted metabolomics results showed that a total of 168 metabolites were identified. The comparison between the groups of BD, vascular involvement in BD and the healthy control group showed that altered amino acid and oxidative stress pathways, especially with glutathione synthesis could be an important stage for developing BD. Conclusion: In the present work, the untargeted metabolomics approach provided new molecular insights for a better understanding of BD pathogenesis and also developing vascular involvement in BD at the metabolite level. The results showed that vascular involvement in BD could be highly linked with amino acid metabolism and also the antioxidant system and these disease-related pathways could be evaluated with further experiments for diagnosis and prognosis of BD and also for vascular involvement in BD.

Background Hypertension is an early finding of autosomal dominant polycystic kidney disease (ADPKD) and is related to different mechanisms. Cyst expansion related renin secretion or early endothelial dysfunctions are some of these hypotheses. Different course of hypertension in ADPKD preoccupies that relatives of ADPKD patients may also be under risk for this underlying mechanisms. In this study, we aimed to find blood pressure response problems to exercise as an initial vascular problem in unaffected relatives of hypertensive ADPKD patients. Methods The cross-sectional study included 24 unaffected relatives (siblings and children) of ADPKD patients and 30 healthy controls that performed a cycle ergometer test. Additionally, as a marker for endothelial function, nitric oxide (NO) and asymmetric dimethylarginine (ADMA) levels at baseline and post exercise were measured. Results Systolic blood pressure (SBP) and diastolic blood pressure (DBP) increases were similar in both groups during the 1st, 3rd, and 6th min of exercise. During the exercise recovery phase SBP decreased in both groups; however, in the relatives of ADPKD patients DBP remained high at the end of the 6th min, suggesting impaired capacity for exercise-induced vasodilatation. Baseline NO and ADMA, and 1-min NO and ADMA were similar in both groups. Conclusion Abnormal blood pressure response to exercise stress was observed in unaffected normotensive relatives. The observed abnormal DBP response pattern to exercise suggests that arterial vascular responses might be already altered in unaffected relatives of hypertensive ADPKD patients; however, long-term clinical trials are needed to clarify the significance of these findings.