AB0791 Risk factors of death of patients with systemic sclerosis (SS) – own observations

Background Nature of SS promotes the development of multiple organ dysfunction and metabolic disorders that are responsible for high mortality. Male gender, subtype dcSSc, the occurrence of aScl-70 and impaired LV systolic function are known risk factors for mortality in SS. Search for new risk factors for death in the SS can improve the prognosis in these patients. Objectives The aim of this study was to demonstrate whether there is a connection between death of SS patients with selected clinical features, serological, and biochemical parameters in the study group. Methods In the study was enrolled 75 SS patients aged 21-75 years treated in the Department of Rheumatology and Internal Medicine of PAM and Rheumatology Outpatient in Szczecin in the years 2004-2010. In the study group six patients died that represent 8.02% of participants. An analysis of the variables that characterize the patients taking into account: sex, SS subtype, duration of disease and Raynaud’s syndrome, NYHA functional classification, interstitial lung disease, cardiovascular diseases (hypertension, ischemic heart disease), nephropathy, and diabetes. The analysis also included: occurrence in patients with SS some autoantibodies (ANA, aScl-70, ACA, AECA), the concentration of acute phase proteins (ESR, fibrinogen, CRP, IL-6), lipid disorders, homocysteine and echocardiographic parameters of dysfunction of systolic and diastolic right and left ventricular hypertrophy. Study was accepted by local ethical commission (BN-001/15/06 and BN-001/143/07). Results Analysis of Causes of Death of Patients with SS Revealed significant associations between: male sex (OR 15.00, 95% CI 2,36-95,47 p=0.004), and dcSSc type of disease (OR 19.50, 95 4,72-913,36% CI, p=0.000), and presence of aScl antibodies (OR 10.32, 95% CI 2,54-485,36, p=0.003) and Stage III of the NYHA (OR 78; 64, 95% CI 2,30-2690,19, p=0.015), and elevated ESR (OR 7.40, 95% CI 1,83-349,32 p=0.001;), and elevated serum level of fibrinogen (OR 42.75, 95% CI 9,82-1998,05 p=0.000;), and elevated serum level of IL-6 (OR 10.88, 95% CI 2,62-528,12, p=0.002), and elevated serum level of Homocysteine (OR 15.18, 95% CI 1,22-205,11 p=0.035;), and lower LVEF (OR 10.99, 95% CI 1,02-118,53 p=0.048) and RVH (OR 16.22, 95% CI 1,40-187,32, p=0.026). Sensitivity and specificity were calculated for each factor of death. It was shown that the highest sensitivity and specificity for risks of death shows: subtype dcSSc (sensitivity 100%, specificity 76, 47%), ESR (sensitivity 100%, specificity 55.22%) and fibrinogen (sensitivity 100%, specificity 76, 47%). However, left ventricular ejection fraction (sensitivity 50%, specificity 91.80%) and homocysteine level (sensitivity 50%, specificity 91.18%) in patients in the study group is characterized by the lowest sensitivity and high specificity. Conclusions 1. Risk factors for death in studied patients with SS are: male gender, subtype dsSSc, aScl, NYHA III degree, accelerated ESR, elevated levels of IL-6, fibrinogen and homocysteine, lower ejection fraction and left ventricular hypertrophy, right ventricle hypertrophy. 2. Among risk factors the highest sensitivity and specificity for mortality show: subtype dcSSc, ESR and fibrinogen concentration, whereas left ventricular ejection fraction and the levels of homocysteine have a lowest sensitivity and high specificity. Disclosure of Interest None Declared