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Introduction Endothelin antagonist receptors (ERAs) and phosphodiesterase-5 inhibitors (PDE5i) are beneficial in pulmonary arterial hypertension (PAH) and digital ulcers (DU) and prevent from DU recurrences. Our study aimed to determine the difference in the incidence rate of PAH and scleroderma renal crisis (SRC) in patients with SSc and DU (SSc-DU) under ERAs/PDE5i or without treatment. Methods We conducted a retrospective cohort study including SSc-DU patients from the Spanish Scleroderma Registry (RESCLE). The primary outcome was the incidence rate of PAH and SRC in patients under ERAs/PDE5i or not. Results Some 544 patients out of 1817 (29.9%) in the RESCLE database had DU, 221 (40.6%) under ERAs/PDE5i and 323 (59.4%) not. The incidence rate (95% CI) difference between patients under treatment or not under did not reach statistical significance in PAH [−0.1 (−4.8, 4.69), P = 0.988] or in SRC [0.7 (−2.2, 3.7), P = 0.620]. However, the time from the first DU to the diagnosis of SRC was delayed in treated patients [mean (s.d.) 7.6 (5.8) years vs 2.9 (5.3); P = 0.021]. The dcSSc subset was more prevalent in the treatment group (36 vs 26%; P = 0.018), along with anti-topoisomerase I antibodies (34 vs 18%; P Conclusion There was no difference in the incidence rate of PAH and SRC between groups. However, treatment with ERAs and/or PDE5i appeared to delay the occurrence of SRC.

The objective of the study is to determine the importance of the mode of onset as prognostic factor in systemic sclerosis (SSc). Data were collected from the Spanish Scleroderma Registry (RESCLE), a nationwide retrospective multicenter database created in 2006. As first symptom, we included Raynaud's phenomenon (RP), cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and digestive hypomotility. A total of 1625 patients were recruited. One thousand three hundred forty-two patients (83%) presented with RP as first symptom and 283 patients (17%) did not. Survival from first symptom in those patients with RP mode of onset was higher at any time than those with onset as non-Raynaud's phenomenon: 97 vs. 90% at 5 years, 93 vs. 82% at 10 years, 83 vs. 62% at 20 years, and 71 vs. 50% at 30 years (p < 0.001). In multivariate analysis, factors related to mortality were older age at onset, male gender, dcSSc subset, ILD, PAH, scleroderma renal crisis (SRC), heart involvement, and the mode of onset with non-Raynaud's phenomenon, especially in the form of puffy hands or pulmonary involvement. The mode of onset should be considered an independent prognostic factor in systemic sclerosis and, in particular, patients who initially present with non-Raynaud's phenomenon may be considered of poor prognosis.

Background Systemic sclerosis (SSc) is a disorder with a well-known autoimmune base. There is evidence suggesting a common background with other connective tissue diseases, thus predisposing to the development of other autoimmne disorders in these patients, that is, poliautoimmunity (PAI). Objectives To assess PAI in a cohort of SSc patients. Methods A nationwide, cross-sectional study was performed including the 1928 patients enrolled in the Spanish SSc Registry (RESCLE). Ethics Committee approval was obtained by all participating centres. Results Prevalence of PAI was 45%, with 32% having more than one association. Most of these patients were women (93%, p We found no significant differences concerning capillaroscopy patterns, or causes of death, neither SSc-related nor non-Ssc-related. Finally, a multivariate analysis with logistic regression was performed to evaluate the risk factors for PAI, which are shown on the table 2. Conclusions PAI in SSc is a frequent condition that special attention in these patients. We found a rather higher prevalence compared with those published in the literature, even though the distribution of those associated disorders was similar. In our cohort, there were no remarkable differences between both groups concerning clinical manifestations (although not always statistically significant). Finally, certain circumstances should make us aware of a possible associated condition to SSc. Reference [1] Elhai M, Avouac J, Kahan A, Allanore Y. Systemic sclerosis at the crossroad of polyautoimmunity. Autoimmun Rev. 2013;12:1052–7. Disclosure of Interest None declared

Background Monotherapy with endotelin antagonist receptors (ERA) an phosphodiesterase 5 (PDE5) inhibitors is a first choice treatment for PAH in functional class (FC) II-III, with the same grade of evidence and recommendation than combination therapy. Recently, studies have proven superiority of combination therapy against monotherapy in combined morbi-mortaliy endpoints. Objectives To demonstrate superiority of combination therapy against monotherapy in a single mortality endpoint in SSc-associated PAH. Methods Retrospective cohort study including patients from the Spanish Scleroderma Registry (RESCLE) diagnosed with SSc-associated-PAH by right heart catheterization (RHC). Patients were divided in 3 groups: monotherapy vs. sequential combination therapy (>12 weeks between first and second treatment) vs. upfront combination therapy ( Results Seventy-six patients with PAH out of 1817 participants were included. Thirty-four (45%)were receiving monotherapy [with ERA (22 patients, 29%) or PDE5 inhibitors (12 patients, 16%)], 25 patients (33%) sequential combination therapy and 17 patients (22%) upfront combination therapy. Baseline demographic, clinical and complementary tests were similar among groups. ILD (mainly moderate) was more frequent in both combination groups in 58% vs. 80% vs. 76.4%, without statistical significance. A worse FVC/DLco in the sequential combination group was reported (2.9±1.1 vs. 1.8±0.4 vs.2.3±0.8, global p=0.085 but p=0.043 comparing monotherapy with sequential combination) and also a worse mPAP in both sequential and upfront combination groups(37.2±8.7 mmHg vs. 40.8±8.8 vs. 46±15.9, p=0.026). The treatment regimen prescribed (p=0.017) and FC at baseline (p=0.007) were found predictors of mortality. Sequential combination therapy was found a protective factor [HR=0.11 (95%CI 0.03–0.51), p=0.004] and the upfront combination therapy showed a tendency of protection [HR=0.68 (95%CI 0.23–1.97), p=0.476]. Survival rates from diagnosis of PAH among groups were: 78% vs. 95.8% vs. 94.1% at 1 year, 40.7% vs. 81.5% vs. 51.8% at 3 years and 31.6% vs. 56.5% vs. 34.5% at 5 years (p=0.007). Side effects were not significantly different among groups. Conclusions Combined sequential therapy improves survival in SSc-PAH patients, even wtih moderate ILD. Upfront combination therapy may improve survival, but did not reach statistical significants due to study limitations. Treatment regimen and FC were found as prognostic factors for survival: sequential combination therapy was a protective factor and FC was a risk factor. Disclosure of Interest None declared

The low overall prevalence of systemic sclerosis (SSc) and the low proportion of male patients have resulted in a scarcity of studies assessing sex differences in Ssc patients, and contradictory results have often been show among those studies that have been performed.A prospective study was conducted with the Spanish RESCLE register to analyse the influence of gender on survival of SSc patients.In total, 1506 SSc patients (1341 women, 165 men) were recruited from 21 centres. Older age at onset (OR 1.02), shorter time from onset to diagnosis (OR 0.96), smoking (OR 2.57), interstitial lung disease (ILD) (OR 1.58), less predisposition to sicca syndrome and to antinuclear antibody positivity (OR 0.29 and 0.43, respectively), and higher compliance with the ACR 1980 criteria (OR 1.79) were independently associated with the male sex. During follow-up, 30.4% of men versus 14.6% of women died (p0.001). Survival at 10 years from the onset of symptoms was 75.3% for men and 92.9% for women (p0.001), and the difference remained after selecting only SSc-related deaths (85.6% vs. 96.1%, p0.001). The mortality predictive factors were diffuse SSc (OR 2.26), ILD (OR 1.82), digital ulcers (OR 1.38), tendon friction rubs (OR 1.74), male sex (OR 1.53), increased age at onset (OR 1.13) and isolated PH (considering only deaths from diagnosis), both in the overall (OR 3.63) and female cohorts (OR 3.97). The same risk factors were observed in the male cohort, except for isolated PH and ILD.The present study confirms the existence of epidemiological, clinical, laboratory and prognostic gender differences in systemic sclerosis patients.