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Background: Pulmonary artery hypertension (PAH, Pulmonary Artery Hypertension) is the most serious cardiopulmonary findings in patients with connective tissue diseases (CTDs, Connective Tissue Diseases) and its early identification is of critical value for proper diagnosis, treatment and final prognosis. PAH is characterized by pulmonary endothelial dysfunction and intense smooth muscle cell proliferation. There are limited data regarding to potent relationship between pulmonary pressure and systemic endothelial function.Aim: was to assess relationship between systemic arteries' vasodilatation and risk for PAH established by standard transthoracic echocardiography (TTE, Transthoracic Echocardiography) in patients with CTDs.Material and methods: 32 patients (K/M: 27/5, age: 55.8 ± 3.1 years) with CTDs (30 - systemic sclerodermia, 2 - systemic lupus erythematosus) were enrolled into the study. Pulmonary fibrosis and extrasystemic causes of pulmonary hypertension were excluded in all patients. Clinical data, TTE parameters, baseline brachial artery diameter (BAd, Brachial Artery diameter), flow-mediated dilatation (FMD, Flow-Mediated Dilatation) and nitroglycerin–mediated dilatation (NMD, Nitroglycerin –Mediated Dilatation) were assessed in all subjects. According to the values of tricuspid regurgitation velocity (TRV, Tricuspid Regurgitation Velocity) and right ventricular systolic pressure (RVSP, Right Ventricular Systolic Pressure) all patients were divided into the three groups: the PH unlikely - group I (n=21), the PH possible – group II (n=7) and the PH likely - group III (n=4).Results: Mean FMD values were comparable between the groups I, II and III (12.6 ± 8.2 vs 11.5 ± 8.6 vs 14.8 ± 6.6%, respectively; p=0.84). The group III showed increased NMD values compared to the group I and II (group I, II, III, respectively : 21.6 ± 8.6 vs 15.0 ± 6.5 vs 41.3 ± 13.9%, p=0.01). Mean values of baseline BAd were as follows: 3.37 ± 0.8 vs 3.11 ± 0.3 vs 2.76 ± 0.8 mm in the groups I, II and III. The BAd values correlated to FMD (r= - 0.68, p=0.03) and NMD (r= -0.71, p=0.02) in the entire examined groups.Conclusions: Systemic endothelial function defined as brachial artery vasodilatation does not correlated to the risk for PAH in patients with CTDs.