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Objective: The aim of this study was to evaluate the exercise tolerance by expired gas analysis during stress test in patients with Systemic Sclerosis (SSc).Methods: Eighteen women (mean age 48.56+/-12.48 years) affected by SSc were studied. A complete echocardiographic examination including pulmonary artery systolic pressure estimation, pulmonary function tests, diffusion lung capacity for carbon monoxide (DLCO), and exercise test were performed. During exercise, breath-by-breath expired gas analysis was performed.Results: Seven patients (39%) had baseline pulmonary systolic hypertension (group A) and 11 patients (61%) did not (group B). Six patients had reduced DLCO values. Both maximal oxygen consumption (VO2max) and anaerobic threshold (VO2AT) values were markedly decreased compared to the predicted values. Seven of 18 patients were unable to complete a maximal exercise (5 of whom affected by pulmonary systolic hypertension). Group A patients showed reduced VO2max, VO2AT, and O2 pulse compared with patients with group B patients (p=0.004, 0.017, and 0.013, respectively); VO2max, VO2AT and O2 pulse were significantly correlated to baseline pulmonary artery systolic pressure.Conclusions: An exercise intolerance in patients affected by SSc is present. Impairment of exercise performance is associated with pulmonary hypertension. [ABSTRACT FROM AUTHOR]

Presents information on a study investigating the relationship of plasma endothelin (ET)-1 levels, pulmonary hypertension and lung fibrosis in patients with systemic sclerosis. ET-1 concentration of patients with systemic sclerosis; Effect of high plasma ET-1 on systemic hypertensive patients.

Investigates behavior of circulating endothelin-1 (ET-1) in patients affected by systemic sclerosis and the relationship between systemic and pulmonary plasma peptide and arterial pressure levels. Plasma ET-1 levels greater in patients affected by systemic sclerosis than in controls; Presence of pulmonary artery systolic hypertension in systemic sclerosis patients.

The diagnosis of skeletal muscle involvement in patients with systemic sclerosis (SSc) is usually based on clinical, laboratory, electromyographic, and bioptic evidence of muscle disorder, whereas SSc cardiac disease is well established by nuclear medicine techniques (radionuclide ventriculography and myocardial scintigraphy). Previous reports have retrospectively hypothesized a possible relationship between cardiac and muscle involvement in scleroderma patients. In order to improve overall diagnostic accuracy in the qualitative/quantitative assessment of skeletal muscle involvement in these patients and to compare these results with those obtained at the cardiac level, diethylenetriaminepentaacetic acid (DTPA)-99mTc radionuclide ventriculography and 99mTc SESTAMIBI myocardial and muscular scintigraphic examinations were performed in 10 SSc patients and in five healthy subjects. Muscular radioactivity, as assessed at thigh and calf levels by means of a segmental score, was significantly decreased in SSc patients in comparison with healthy subjects (global score value 15.6 ±2.2 vs 22.7 ±1.6, p<0.001), as well as right ventricular ejection fraction (RVEF, 34.3% ±5.3 vs 53.6% ±4.2, p<0.001) and myocardial segmental perfusion (global score value, 19.6 ±2 vs 25.9 ±1.1, p<0.01). The results show a high frequency of skeletal muscle involvement in patients with SSc. Moreover, scleroderma patients with muscle disorders, as evidenced by scintigraphy, show a comparable occurrence of cardiac involvement, even in the absence of clinical signs of cardiac dysfunction. [ABSTRACT FROM AUTHOR]