AB0791 Risk factors of death of patients with systemic sclerosis (SS) – own observations
Authors: B. Trzcinska-Butkiewicz, M. Peregud-Pogorzelska, M. Wόdecki, M. Brzosko
Journal: Annals of the Rheumatic Diseases (2013)

<h3>Background</h3> 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. <h3>Objectives</h3> 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. <h3>Methods</h3> 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). <h3>Results</h3> 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. <h3>Conclusions</h3> 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. <h3>Disclosure of Interest</h3> None Declared