Pulmonary hypertension (PH) represents rare and serious complication of various systemic connective tissue diseases. It occurs most frequently in scleroderma, mixed connective tissue disease, systemic lupus erythematosus and in antiphospholipid syndrome. PH in systemic diseases is newly classified as the arterial PH. Its development is potentiated by several epigenetic mechanisms including vasculitis, vasculopathy, thromboembolia, vascular endothelial impairment, and by the imbalance between vasoconstriction and vasodilatation mechanisms. Suspection of PH can be established on the basis of echocardiography and the diagnosis is verified by right-side catheterisation. The degree of reversibility of vasoconstriction of pulmonary vessels and therefore the most suitable therapy can be estimated using vasodilatation test. PH treatment has recently undergone a rapid development. Formerly, calcium channel blockers were used and since the end of eighties patients have been treated with prostacycline and epoprostenole respectively, administered in continuous intravenous infusion. In recent few years some other drugs were tested, namely the epoprostenole derivatives. These drugs need not be administered only intravenously; they can be given per inhalation (iloprost), subcutaneously (treprostinol) or perorally (beraprost). Another new drug is the phosphodiesterase inhibitor (sildenafil), endothelial receptor blocker (bosentan), nitrogen oxide or L-arginine and also therapy using adenosine and serine is being tested. Some combinations of drugs are also examined. The above given possibilities of treatment, however, are not routinly available, they can be tested only within the framework of therapeutical research studies. PH treatment is life lasting and comparatively costly. The therapy of the systemic connective tissue disease has no effects on the already developed PH, but it is necessary for stabilization of the primary disease.

        Key words: pulmonary hypertension, systemic lupus erythematosus, antiphospholipid antibodies, heart failure, vasodilatation therapy