| Popis: |
Background Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by microvascular abnormalities. Cardiopulmonary and vascular manifestations in SSc are the most important survival factors in the disesase. Objectives Correlations between different cardiovascular parameters (ejection fraction (EF), diastolic dysfunction, wall motion disturbance, estimated systolic pulmonary arterial pressure (sPAP), flow-mediated vasodilatation, arterial stiffness parameters (augmentation index - aIx, pulse wave velocity - PWV) and disease outcome and death were measured. Methods Thirty-six SSc patients were involved who underwent detailed arterial stiffness and vasodilatation examinations in 2007. In our work a 10-year period was analysed till 2017 following the progression of organ manifestations and survival. Results During the 10-year follow-up, 13 of the 36 patients died. The average survival time was 26.59 (22.36-37.64) years. Significant correlation was found between the initial aIx and PWV with cardiac wall motion disturbance (p = 0.018 and p = 0.016), aIx with restrictive ventilation dysfunction (p = 0.029) and PWV with obstructive ventilation dysfunction (p = 0.015). During the 10-year period, the occurrence of diastolic dysfunction was significantly increased (p = 0.0016), the incidence of arrhythmia did not change significantly. A significant risk factor for survival has been the decrease in EF (p = 0.011), where 1% reduction in EF was associated with 1.9% increase in mortality and 1 mmHg increase in sPAP involved 13% increase in mortality. Conclusion Based on our findings vascular stiffness parameters can be the predictors not only of the cardiovascular mortality but pulmonary manifestations with restrictive ventilation disturbances also in SSc. Disclosure of interests Mariann Kalaszi: None declared, Renata Laczik: None declared, Pal Soltesz: None declared, Katalin Hodosi: None declared, Szilvia Szamosi Speakers bureau: Roche, Zoltan Szekanecz Grant/research support from: Pfizer, UCB, Consultant for: Pfizer, abbvie, Roche, Sanofi, Lilly, Novartis, Speakers bureau: Pfizer, abbvie, Roche, Sanofi, Lilly, Novartis, Gabriella Szucs Speakers bureau: actelion, Roche, Sager |
