endothelin-1 and Takayasu-Arteritis

To investigate the probable pathogenesis, clinical features, diagnosis, and therapy of patients with pulmonary hypertension (PH) in Takayasu arteritis (TA).. A total of 48 patients with TA who had PH, 20 patients with TA who had pulmonary arterial involvement (PA) without PH, and 30 patients with idiopathic pulmonary arterial hypertension (IPAH) were enrolled in the study from 2009 to 2013.. Among the 48 patients with TA who had PH, 36 (75.0%) had PA, and left heart disease (LHD) was present in 12 (25.0%). Serum levels of big endothelin 1 (ET-1) were independently correlated with pulmonary arterial systolic pressure (r = 0.33, p = 0.04). Compared to patients with IPAH, patients with PH because of PA who underwent right heart catheterization had lower average cardiac indexes (2.0 ± 0.5 vs 3.0 ± 1.2 l/min/m(2), p = 0.05), and they all developed favorable responses to acute vasodilator testing (100%) in comparison to 10 of the patients with IPAH (33.3%). During a mean followup of 36.0 ± 13.2 months (12.0-65.0 mos), of the patients with PH associated with PA, 3 died of heart failure. Six patients who underwent pulmonary artery revascularization were found to have good prognoses after followup for a mean duration of 6.2 ± 1.9 months. Additionally, 12 patients with PH with LHD were followed for 38.4 ± 15.6 months (12.0-60.0 mos), and 1 patient died of heart failure during the followup period.. Patients with TA are at increased risk for PH. Early screening of patients with TA with unexplained symptoms related to PH should be applied. PH-specific therapies or revascularization may be effective treatments in the early stages of patients with PA, PH, and severe pulmonary artery stenosis.

Topics: Adolescent; Adult; Cardiac Catheterization; Endothelin-1; Female; Humans; Hypertension, Pulmonary; Male; Middle Aged; Prognosis; Takayasu Arteritis; Treatment Outcome; Young Adult

The objective of this study was to evaluate traditional risk factors for cardiovascular disease (CVD) and endothelin-1 (ET-1) levels in Takayasu arteritis (TA) patients. Twenty-two TA patients and 37 controls were evaluated. TA patients had a higher prevalence of hypertension (63.6% vs. 21.6%, p=0.001) and higher levels of triglycerides (129.5 mg/dL+/-70.8 vs. 88.4 mg/dL+/-60.8, p=0.017) than controls. Mean number of CVD risk factors was 1.64+/-1.22 in TA patients and 1.03+/-1.44 among controls, p=0.030. More TA patients presented at least one CVD risk factor when compared to controls (77.2% vs. 51.3%, p=0.048). ET-1 levels were higher in patients than in controls (1.49 pg/mL+/-0.45 vs. 1.27 pg/mL+/-0.32, p=0.034), however no significant difference was found between patients with active and inactive disease. In this study, TA patients presented a higher prevalence of hypertension, higher levels of triglycerides, and ET-1 than controls.

Topics: Adult; Atherosclerosis; Cardiovascular Diseases; Endothelin-1; Female; Humans; Hypertension; Inflammation; Male; Middle Aged; Risk Factors; Takayasu Arteritis; Treatment Outcome; Triglycerides

Prognosis in Takayasu's arteritis is limited owing to renovascular hypertension. The authors report a patient with Takayasu's arteritis who had been unilaterally nephrectomized and presented with malignant hypertension due to renal artery stenosis. Hypertension was refractory to conventional antihypertensive treatment, and stenosis was not accessible by interventional angioplasty. Initiation of enalapril and losartan therapy was successful in improving blood pressure without deterioration of renal function due to ischemic failure. Antihypertensive treatment resulted in dramatically stimulated endogenous nitric oxide (NO) synthesis, while elevated plasma endothelin-1 levels were unchanged. Renovascular hypertension in Takayasu's arteritis is associated with an imbalance of vasoconstrictor peptide endothelin-1 and vasodilator peptide NO. Successful treatment of hypertension by enalapril or losartan results in improved endogenous NO synthesis, which putatively counterbalances excessive vasoconstrictor actions and may retard the progression of renal failure.

Topics: Angiotensin-Converting Enzyme Inhibitors; Antihypertensive Agents; Enalapril; Endothelin-1; Female; Humans; Hypertension, Renovascular; Losartan; Middle Aged; Nephrectomy; Nitric Oxide; Prognosis; Renal Artery Obstruction; Takayasu Arteritis; Vasoconstrictor Agents; Vasodilator Agents