Progressive systemic sclerosis (PSS) is characterized in its first phases by vascular damage. Lungs are involved in two thirds of patients with initial progressive destruction of the capillary bed and consequent reduction of the functional reserve, which may lead to hypertension of the pulmonary circulation. For these reasons it is of great interest to have early information about the pressure of the pulmonary circulation, both at rest and during exercise, to follow the progression and the evolution of the illness independently from subjective symptoms. The aim of the study was to evaluate by a noninvasive method, saline-enhanced Doppler echocardiography, the behavior of the right ventricular systolic pressure in patients with PSS, at rest and during exercise, without clear instrumental or clinical signs of pulmonary involvement at rest. Nine patients (7 women and 2 men) with PSS, aged 55.7 +/- 8.7 years, and 9 control subjects were evaluated. All patients had normal pulmonary pressure at rest and negative history for effort dyspnea. Subjects underwent Doppler echocardiographic examination at rest and during exercise. Right ventricular systolic pressure was evaluated by saline-enhanced Doppler technique, at rest and throughout exercise. At rest the right ventricular systolic pressure was normal in all patients and controls. At the end of exercise, in 4 patients, values were still normal (40.7 +/- 2.2 mmHg); in the others pathologic values were recorded (59.8 +/- 3.9 mmHg). In the control group values were always normal (35.6 +/- 4.6 mmHg). In our study the saline-enhanced Doppler echocardiography has been demonstrated to be an important diagnostic tool for the noninvasive evaluation of right ventricular systolic pressure, both at rest and during exercise; it could be useful in monitoring the pulmonary vascular damage in patients with PSS.