Abstract
Systemic scleroderma is a disease of connecting tissues, characterized by functional, later structural hypertension. A case of a 64-year-old patient is described who started to feel congealing of the skin in his fingers in 2005, which spread later to the face and body.
On the grounds of the American College of Rheumatology (ACR) criteria from 1980, SSc was diagnosed, and clinically classified as a diffuse form. Searching for organ manifestations by means of functional lung examination, the ratio of appropriate values of vital capacity and the diffuse lung capacity for CO (FVC/DLCO) was 1.75.
Ultrasonographic (US) examination of the heart revealed pulmonary hypertension with estimated systolic pressure of 75 mmHg in the pulmonary artery. The catheter blood pressure in PA was defined as 87/48/61 mmHg and in the impacted pulmonary bloodstream 21 mmHg.
The condition was assessed as heavy PAH associated with SSc and bosentan medication was commenced. Recommended are regular US of the heart once a year.