Although more common in women, SSc appears as strikingly more severe in men. Our results obtained through the largest worldwide database demonstrate a higher risk of severe cardiovascular involvement in men. These results raise the point of including sex in the management and the decision-making process.

Background Systemic sclerosis (SSc) is associated with a significant reduction in survival in comparison to the general population. This is the first report on 5-year survival and its predictors in a Romanian cohort of SSc patients. Objective We aimed to assess the 5-year survival rate in a single-center cohort of SSc patients and to identify predictors of death and disease worsening. Methods All patients of the EUSTAR Center 100 enrolled before 2009 and who had at least 2 visits at a minimum interval of 5 years, or who died after at least 3 months of follow-up, were included. All patients were assessed according to current EUSTAR recommendations. A comparison was made between the surviving and the deceased patients regarding all MEDS baseline parameters. Using age-adjusted univariate logistic regression we identified predictors for death and for several outcomes considered as disease worsening: 20% reduction in forced vital capacity (FVC); 20% reduction in diffusing capacity of the lung for carbon monoxide (DLCO); development of pulmonary arterial hypertension (PAH) as assessed by power Doppler heart ultrasound; and digital ulcers (DUs) recurrent at prospective visits. Results Out of 68 patients enrolled before 01.01.2009, 40 met the inclusion criteria (82,5% females, 55% limited cutaneous subset, mean ± SD follow-up period 5.7 ± 2.1 years; mean ± SD age at first visit 49 ± 11.8 years; mean ± SD disease duration at first visit 4.4 ± 6.0 years). Throughout the 5-year follow-up period there were 7 deaths (including 3 SSc-related deaths), resulting in an overall 5-year survival rate of 82,5%. In survivors, lung function tests deteriorated significantly in 11% (FVC) and 52% (DLCO), while 20% developed PAH. Recurrent or newly appearing DUs occurred in 21% of all 42 patients. Significant predictors for death were the diffuse cutaneous subset, and the presence of DUs at presentation, with odd ratios [95% confidence interval] (OR[CI95%]) of 14,2 [1.3-151] and 38 [2.9-501] respectively. Conduction blocks on the baseline ECG predicted PAH with an OR [95% CI] of 12.8 [1.5-108]. Significant predictors for DUs were active DUs and calcinosis at presentation: OR [CI95%] 5.2 [1.1-26] and 6 [1.1-33] respectively. None of the parameters tested as potential predictors of the respiratory function decline achieved statistical significance. Conclusions The survival rate in our cohort was 82.5%, which is similar to other cohorts from developed countries. We identified diffuse cutaneous subset, active digital ulcers, and heart conduction blocks as predictors for a poor disease outcome.

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BackgroundSystemic sclerosis (ScS) is a polymorphic autoimmune disease, with increased morbidity and mortality, often diagnosed in advanced stages. The recently published 2013 American College Of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for SSc were developed to increase the identification of early/mild SSc patients.ObjectivesTo compare the sensitivity and specificity of the 2013 ACR/EULAR criteria with 1980 ACR criteria in a single-centre cohort of patients with SSc, diagnosed according to expert oppinion.MethodsData of SSc patients evaluated in our EUSTAR centre between 2004–2015 have been retrospectively analyzed. The control cohort included patients with Raynaud's phenomenon of other ethiology than SSc, evaluated between 2009–2015. The sensitivity and specificity of the 2013 ACR/EULAR and 1980 ACR criteria were tested using the McNemar test for correlated proportions.ResultsOne hundred and fifty one patients with SSc (90.1% women, mean±SD age 58.4±13.2 years) and 65 controls (87.7% women, mean±SD age 43.7±14.6 years) were included. The sensitivity of the 2013 ACR/EULAR and 1980 ACR criteria were 94.7% and 84.4%, respectively (p<0.001). The specificity of the 2013 ACR/EULAR and 1980 ACR criteria were 86.2% and 96.9%, respectively (p=0.01). Patients missclassified according to the new 2013 ACR/EULAR criteria were 17, whereas according to the old 1980 ACR criteria were 32. The sensitivity of the 2013ACR/EULAR was significantly better than the 1980 ACR criteria in the limited cutaneous SSc patients (93.1 vs. 82.2%, p<0.001).ConclusionsThe 2013 ACR/EULAR criteria have a better sensitivity and missclassify fewer patients than the 1980 ACR criteria. Their excelent performance is close to the gold standard of diagnosis in SSc (expert opinion).Acknowledgement*This abstract was realized as part of the “Development of a computer-based nailfold videocapillaroscopy (NVC) system for longitudinal evaluation of patients with systemic sclerosis” (QUANTICAP) project, financed by the UEFIS-CDI PN-II-PT-PCCA-2013–4-1589 grant.Disclosure of InterestNone declared