SURVIVAL OF PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION, ASSOCIATED WITH SYSTEMIC SCLEROSIS

Aim. To study survival of patients with a natural course of pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SS). Material and methods. Twenty SD patients with PAH and 71 SS patients free of PAH were followed up for 10 years (1998-2008) when therapy with specific pulmonary vasodilators was not available. Survival curves for both groups were plotted by Kaplan-Mayer method. Results. One-, 3and 5-year survival were 85, 25 and 5%, respectively, in the SS-PAH group and 99, 82 and 77% in the SS group, respectively. The differences were significant (р < 0,001). Survival median in the group SS-PAH was 32 months. Conclusion. The above evidence is comparable to the results obtained earlier and demonstrate a fatal outcome of the disease in the absence of therapy with specific pulmonary vasodilators.