Effect of sildenafil on clinical evidence and hemodynamic parameters in patients with pulmonary arterial hypertension, associated withsystemic rheumatic diseases

Timeliness of the topic: Pulmonary arterial hypertension(PAH), associated with systemic rheumaticdiseases(SRD),in particular with diffuse scleroderma, is a progressive disorder characterized by adverse outcomeespecially in lack of adequate treatment. Sildenafil 20 mg relaxes blood vessels and shows antiproliferative effect in patients with SRD.The aim of this paperis to study efficiency and safety of sildenafil in patients with PAH, associated with systemic rheumatic diseases.Material and methods:we have been monitoring the 16-week therapyin 14 patients with PAH, associatedwith systemic scleroderma (12 patients) and systemic lupus erythematosus (2 patients). The efficiency was assessed by the dynamics of clinico-laboratorial indices, central hemodynamics were evaluated using the right heart catheterization before the study and after 16 weeks of treatment.Results:We marked thatthe walkeddistancehad been increased by 31 meterinsix-minute walking testduring the therapy;we also saw the improvement of thefunctional classin 29% of patients.The significant improvement achieved in evaluating the hemodynamic parameters.A significant reduction of mean pulmonary arterial pressure was determined from 50.4 ± 9.2 mm Hg to 40.7 ± 10.2 mm. (p = 0.002), the pulmonary vascular resistance fell from 9.5 ± 5.9 to 6.4 ± 3.7 Wood units(p = 0.05),as well assome of the other indicators decreased.Right atrial pressurefell from8.3 ± 5.5 mm Hg. to 6.5 ± 3.1 mm Hg,we noticedthe stabilizationof left ventricle systolic function indices, thus,cardiac indexin all 14 patients,participatingin our study,had not been undergoingsignificant changes (3.1 ± 0.9 l/min/m2at the beginning of the study and 3.1 ± 0.7 l/min/m 2after 16 weeks of the therapy).The risk level, using scale register REVEAL, significantly decreased frommoderate high (9.0 ± 2.0)to medium (8.2 ± 1.9), (p = 0.04).There were no serious adverse events during the 16-week treatment using sildenafil. Three patients showed predisposition to hypotension, but, the blood pressuredecreased within the normal rangesin total group. Skin changes characterized bydermahemia ("blush") were observed in 4 patients and did not significantly impact on the general well-being of patients.Conclusion: Thus, Sildenafil 20 mgshowedefficiency and safety and could be used in treatment patients with PAH, associated with SRD.