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.

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Objectives The prevalence and characteristics of systemic sclerosis-associated interstitial lung disease (SSc-ILD) vary between geographical regions worldwide. The objectives of this study were to explore the differences in terms of prevalence, phenotype, treatment, and prognosis in patients with SSc-ILD from predetermined geographical regions in the EUSTAR database. Methods Patients were clustered into seven geographical regions. Clinical characteristics and survival of patients with SSc-ILD were compared among these pre-determined regions. Results For baseline analyses, 9260 SSc patients were included, with 6732 for survival analyses. The prevalence of SSc-ILD in the overall population was 50.2%, ranging from 44.0% in “Western Europe & Nordic countries” to 67.5% in “Eastern European, Russia & Baltic countries”. In all regions, anti-topoisomerase antibodies were associated with SSc-ILD. Management also significantly differed; mycophenolate mofetil was prescribed at baseline in 31.6% of patients with SSc-ILD in “America (North & South)” and 31.7% in “Middle East” but only 4.3% in “Asia & Oceania” (P < 0.0001). Patients from “America (North & South)” and “Middle East” had the highest survival rate at the end of follow-up (85.8% and 85.2%, respectively). Conclusion Our study highlights key differences among regions in terms of clinical presentation and prognosis of SSc-ILD. This work also demonstrates that the management of SSc-ILD is highly variable among the different regions considered, suggesting that efforts are still needed for the standardisation of medical practice in the treatment of this disease.

Objective To develop and validate the prognostic prediction model DU-VASC to assist the clinicians in decision-making regarding the use of platelet inhibitors (PIs) for the management of digital ulcers in patients with systemic sclerosis. Secondly, to assess the incremental value of PIs as predictor. Methods We analysed patient data from the European Scleroderma Trials and Research group registry (one time point assessed). Three sets of derivation/validation cohorts were obtained from the original cohort. Using logistic regression, we developed a model for prediction of DUs. C-statistics and calibration plots were calculated to evaluate the prediction performance. Variable importance plots and the decrease in C-statistics were used to address the importance of the predictors. Results Of 3710 patients in the original cohort, 487 had DUs and 90 were exposed to PIs. For the DU-VASC model, which includes 27 predictors, we observed good calibration and discrimination in all cohorts (C-statistics = 81.1% [95%CI: 78.9%-83.4%] for the derivation and 82.3% [95%CI: 779.3%-85.3%] for the independent temporal validation cohort). Exposure to PIs was associated with absence of DUs and was the most important therapeutic predictor. Further important factors associated with absence of DUs were: lower mRSS, anti-Scl-70 negativity and normal CRP. Conversely, the exposure to PDE-5i, prostacyclin analogues or ERAs seemed to be associated with the occurrence of DUs. Nonetheless, previous DUs remains the most impactful predictor of DUs. Conclusion The DU-VASC model, with good calibration and discrimination ability, revealed that PIs treatment was the most important therapy-related predictor associated with reduced DUs occurrence.

Background Gastrointestinal involvement in systemic sclerosis (SS) is frequent (70-90% of the patients), different (it affected the whole digestive system) and persistent, contributing to the alteration of quality of life in the patients. In order to prevent severe complications of this manifestation it is necessary to diagnose early and initiate the adequate treatment. Objectives Studying the variants of presentation of gastrointestinal involvement in systemic sclerosis patients and finding their dependence on the clinical forms of the disease. Methods The study comprised 105 patients with SS, out of which only 6 were male. The mean age of the study group was 44,2 years. The mean disease duration – 14,3 years. Out of them, 78 (74,3%) patients displayed a limited form of disease and 27 (13,3%) patients had diffuse injury. We have used ARA criteria (1980) as diagnostic tools. The patients were followed up during 5 years (2007-2012). They were comprehensively examined clinically and paraclinically, inclusively fibroesophagogastroduodenoscopia (FEGDS), X-ray examination with barium contrast of esophagus and bowel, microbiological examination of the stool. Results The frequency of gastrointestinal involvement in the studied patients was 84,8% (89 persons). According to the patients’ complaints they were devised as following: the dry mouth-31,5%, microstomia-23,6%, heavy teeth problems-19,1%, disfagia-71,9%, heartburn-53,9%, early empting of stomach-36%, nausea-16,9%, distention/bloating-50,6%, abdominal pain-50,6%, recurrent diarrhea-25,8%, constipation-46,1% (41 patients), anal sphincter incontinence-4,5%, (4 patients), significant weight loss-33,7% (30 patients). According to detected disorders on imagistic examination: esophageal hypomotility, observed on X-ray examination with barium contrast-66,3 % (59 patients), esophageal dilatation detected on chest computer tomography-29,2 % (26 patients), esophagitis detected on FEGDS -15,7% (14 patients), the presence of gastric antral vascular ectasia-4,5% (4 patients), bowel hypo motility was observed on X-ray examination with barium contrast-32,6% (29 patients), malabsorption syndrome-3,4% (3 patients), primary biliary cirrhosis-1,1% (1patient). During the observation died 4 patients (4,5%), among them 1 (1,1%) died of severe malabsorbtion. Analyzing the dependence variants of digestive involvement on clinical form of the disease we observed that in diffuse form is present diarrhea (77,8% versus 2,6%, p<0,01), malabsorption syndrome (11,1% versus 0%, p<0,01), weight loss (70,4% versus 14,1%, p<0,01), but in limited form – disfagia (66,7% versus 44,4%, p<0,05), heartburn (50% versus 33,3%, p<0,05), constipation (49,7% versus 11,1%, p<0,01), anal sphincter incontinence (5,1% versus 0%, p<0,05). Conclusions Digestive involvement in patients with systemic sclerosis is frequent, diverse and complex. Patients with diffuse form frequently develop diarrhea, malabsorbtion syndrome and, weight loss, but those with limited form – disphagia, heartburn, constipation and anal sph...