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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.

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Background:Concomitant presence of two autoimmune diseases, such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) is known as “Rhupus”. Although poliautoimmunity is not uncommon phenomenon, only a small series of patients have been described so far with Rhupus.Objectives:Our purpose was to analyze the clinical and serological characteristics of patients with Rhupus and compare them with a cohort of patients with SLE.Methods:In this cross-sectional study, we included cases of Rhupus (ACR/EULAR 2010 plus ACR 1987 criteria) from 11 different Rheumatology Departments at Catalonia, Spain. We included patients with a diagnosis of SLE in a 2:1 ratio matched by sex, race and disease duration. To avoid misclassification, those patients with Rhupus but who had Jaccoud’s arthropathy or with overlap syndromes were excluded.Results:A total of 120 patients were included, 40 cases with Rhupus and 80 cases with SLE. Most of patients were female (95%) and Caucasian (75%). Mean age was 51.0 ± 14.7 years with a mean disease duration of 12.9 ± 9.2 years. Main clinical characteristics were articular involvement (93.3%), cutaneous involvement (77.5%), haematological (72.5%), secondary Sjögren syndrome (38.7%) among others. Clinical and serological characteristics according different groups are shown in Table. Total N= 120 Rhupus n = 40 SLE n = 80 Gender (Female),%114 (95%)38 (95%)76 (95%) 1Mean age, years ± SD51,0 ± 14,757,10 ± 14,1§ 47,9 ± 14,2Disease duration, years ± SD12,9 ± 9,213,6 ± 7,912,6 ± 9,8Race (Caucasian),%84 (75%)25 (78,1%)59 (73,8%) Clinical characteristics Cutaneous involvement,%93 (77,5%)31 (77,5%)62 (77,5%)Articular involvement,%112 (93,3%)40 (100%) § 72 (90,0%)· Arthritis,%96 (80,0%)40 (100%) §§ 56 (70,0%)· Erosive disease,%25 (20,8%)24 (60,0%) §§ 1 (1,3%)· Tenosynovitis,%37 (30,8%)22 (55,0%) §§ 15 (18,8%)Renal involvement,%24 (10%)4 (10,0%) § 20 (25,0%)Mean SLEDAI *2,9 ± 2,82,6 ±2,53,0 ± 3,0 Immunological features Positive RF,%37 (32,7%)32 (80%) §§ 6 (6,8%)Positive anti-CCP,%33 (30,6%)31(81,6%) §§ 2 (2,9%)Comorbidity ¥ 44 (36,7%)20 (50,0%) § 24 (30,0%)SLICC *0,8 ± 1,361,3 ± 1,55 §§ 0,6 ±1,2 Treatment (ever) Prednisolone,%100 (84,7%)39 (97,5%) §§ 61 (78,2%)Antimalarial109 (91,6%)35 (89,7%)74 (92,5%)MTX%58 (48,7%)33 (82,5%)§§ 25 (31,6%)Rituximab,%24 (20,2%)13 (33,3%)§ 11 (13,8%)* Last visit, ¥ Comorbidities included: Hypertension, DM, dyslipemia, osteoporosis or Cushing.Conclusion:We found some clinical and serological differences among patients with Rhupus vs SLE alone. As expected, articular domains and positive RF and ACPAs were higher in Rhupus. By other hand, renal involvement was more common among “pure” SLE patients. Rhupus patients were more commonly treated with prednisolone, MTX and rituximab, and had more comorbidities and organ damage. If Rhupus represent a different condition, requires further analysis in bigger cohorts.Disclosure of Interests:Beatriz Frade Sosa: None declared, J. Narváez Consultant for: Bristol-Myers Squibb, Tarek Carlos Salman Monte: None declared, Vera Ortiz Santa...