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Background/Purpose Systemic lupus erythematosus (SLE) is an autoimmune disease, mostly affecting women during their childbearing age. Disease remission before conception is pivotal for a favorable pregnancy outcome. The aims of our study were: 1)to evaluate maternal and fetal complications in a cohort of pregnant SLE patients; 2)to compare Systemic Lupus Erythematosus Disease Activity Score (SLE-DAS) and Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) in the 6 months before conception as predictors of maternal/fetal complications. Methods All SLE pregnancies were prospectively included until August 2019 at our Unit (Rheumatology Department, University of Padova). All women were monthly followed throughout gestation and at least in one occasion in the 6 months before conception. Disease activity was assessed before pregnancy by SLEDAI-2k and SLEDAS. During gestation, we evaluated SLE activity by the Systemic Lupus Erythematosus Pregnancy Disease Activity Index (SLEPDAI) and the SLEDAS index adapted to pregnancy (SLEPDAS). At conception, we considered ‘active’:1)patients with a SLEDAS>2.08; 2)clinically active patients according SLEDAI-2k treated with ≥5 mg/day of prednisone (immunosuppressants and antimalarials were allowed). Statistics were performed with SPSS (V.24.0). Results We enrolled 115 pregnancies in 76 patients (table 1). We found 29 (25.2%) pregnancies with at least one maternal complication and 39 (33.9%) with at least 1 fetal adverse outcome. Positive anti-dsDNA and active disease according SLEDAS (SLEDAS>2.08) at conception resulted to be predictors of maternal complications (p=0.036, OR=2.71, CI 95% 1.07–6.87; p=0.008, OR=4.46, CI 95% 1.48–13.49, respectively). Conversely, active disease according SLEDAI-2k did not result predictive of maternal complications (p=0.255, OR=2.16, CI 95% 9.57–8.16). No predictors of fetal complications were observed. Conclusions SLE-DAS>2.08 and positive anti-dsDNA are risk factors of maternal complications while active SLE according to SLEDAI-2k does not seem to be a predictor of such complications.

Background Belimumab is the unique biologic therapy available for patients with SLE. Objectives To investigate effectiveness and safety of belimumab in SLE patients in clinical practice. Methods 458 active SLE patients (ACR criteria) from 24 Italian Centers, mean±SD age 43.5±11.3 years; mean±SD disease duration 12.3±8.7 years, were treated with belimumab (10 mg/kg day 0, 14, 28 and then every 28 days), as add-on therapy. SLEDAI-2K, anti-dsDNA, C3, C4, prednisone daily dose, DAS-28, 24H proteinuria, CLASI, PGA, Fatigue (VAS 0-10) were recorded at baseline and every 6 months. Flares were defined according to SFI. Response was evaluated according to SRI-4. Statistics were performed by pairs T-test, chi-square test and multiple logistic regression (SPSS, version 22.0). Results Mean±SD follow-up was 21.2±15.3 months (range 3-60). Most common features treated with belimumab were articular in 67%, mucocutaneous in 55%, and renal in 17% of cases. Improvement of clinical and serological variables, including daily prednisone dosage, was observed (Table). SRI-4 is summarized in the Figure. At the end of follow-up 293 patients (66%) were still on belimumab. Most common cause of discontinuation were inadequate response (36%), AEs (31%), and pregnancy (8%). Mean number of flare during belimumab treatment compared with the corresponding period before belimumab initiation decreased (p 9,998 infusions were analyzed. 784 AEs were observed in 330 patients, SAEs were 43 in 36 patients. No severe infusion reactions were observed; 16 patients had infective SAEs, and 22 non infective SAEs. Conclusion We confirmed the effectiveness, the steroid sparing effect and good safety profile of belimumab in our cohort. Disclosure of Interests Luca Iaccarino: None declared, Francesca Saccon: None declared, Alessandro Mathieu: None declared, Matteo Piga: None declared, Angela Ceribelli: None declared, Carlo Selmi Grant/research support from: Abbvie, Janssen, MSD, Novartis, Pfizer, Consultant for: Abbvie, Alfa-Sigma, Biogen, BMS, Celgene, Eli-Lilly, GSK, Janssen, Merck Sharp and Dohme, Novartis, Pfizer, Roche, Sanofi-Genzyme, YCB, Speakers bureau: Abbvie, Alfa-Sigma, Biogen, BMS, Celgene, Eli-Lilly, GSK, Janssen, Merck Sharp and Dohme, Novartis, Pfizer, Roche, Sanofi-Genzyme, YCB, Paolo Cardinaletti: None declared, Armando Gabrielli: None declared, Andrea Di Matteo: None declared, Rossella De Angelis: None declared, Paola Faggioli: None declared, Antonella Laria: None declared, Micaela Fredi: None declared, Francesca Regola: None declared, Laura Andreoli: None declared, Giulia Pazzola: None declared, Carlo Salvarani: None declared, Francesco Puppo: None declared, Simone Negrini: None declared, Marcella Prete: None declared, Vito Racanelli: None declared, Elisa Gremese Consultant for: AbbVie, BMS, Celgene, Janssen, Lilly, MSD, Novartis, Sanofi, UCB, Roche, and Pfizer, Speakers bureau: BMS, Speakers bureau: Roche, Speakers bureau: AbbVie, BMS, Celgene, Janssen, Lilly, MSD, Novartis, Sanofi, UCB, Roche, and Pfizer, Maria Gerosa: None declared, Tania Ubiali: None declared, Enrica Bozzolo: None declared, Valentina Canti: None declared, Fabrizio Conti: None declared, Fulvia Ceccarelli: None declared, Elena Bartoloni Bocci: None declared, Roberto Gerli: None declared, Antonio Lobasso: None declared, Amato De Paulis: None declared, Ginevra De Marchi: None declared, Salvatore De Vita Grant/research support from: Roche, Pfizer, Abbvie, Novartis, BMS, MSD, Celgene, Janssen, Consultant for: Roche, Alessandra Bortoluzzi: None declared, Marcello Govoni Paid instructor for: Pfizer, Roche, Speakers bureau: Pfizer, Abbvie, MSD, Roche, Eli-Lilly, Celgene, Sanofi, Janssen, Francesco Benvenuti: None declared, Margherita Zen: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB, Chiara Tani: None declared, Maurizio Rossini: None declared, Giovanni Orsolini Speakers bureau: Grunenthal, Mariele Gatto: None declared, Salvatore Scarpato: None declared, Enrico Brunetta: None declared, Aurora Zumbo: None declared, Gabriele Valentini: None declared, SERENA FASANO: None declared, Giacomo Emmi: None declared, Maria Letizia Urban: None declared, Giacomo Tanti: None declared, Angela Tincani Consultant for: UCB, Pfizer, Abbvie, BMS, Sanofi, Roche, GSK, AlphaSigma, Lillly, Jannsen, Cellgene, Novartis, Andrea Doria: None declared

Background: The treat-to-target strategy in Systemic Lupus Erythematosus (SLE) aims to achieve remission. However, to define a target based on the SLE Disease Activity Index (SLEDAI) is questionable, due to its limitations (especially its dichotomous nature). The SLE Disease Activity Score (SLE-DAS) is a recently validated continuous disease activity score which has a higher accuracy in measuring SLE activity and a higher sensitivity-to-change compared to SLEDAI.1 Objectives: To assess the ability of SLE-DAS to define SLE remission and other disease activity states. Methods: Cross-sectional study of SLE patients fulfilling the ACR’97 and/or SLICC’12 classification criteria and followed at the Padua Lupus Clinic from March to June 2018. At each outpatient visit, the attending clinician scored SLE disease activity (in the last 30 days) using Physician Global Assessment (PGA) (0-3 points, 10 cm scale), SLEDAI-2K and SLE-DAS. A senior rheumatologist expert in SLE, blinded to the disease activity scores, classified each patient in 1 of 4 categories: (i) remission, (ii) low disease activity (LDA), (iii) mild disease activity and (iv) moderate/severe disease activity. The best cut-off values of SLE-DAS to define these categories were estimated using Receiver Operating Characteristic (ROC) curve analysis. Accuracy, precision, sensitivity and specificity values for these cut-off values were then calculated. The agreement between the SLE-DAS and physician’s classification was measured using Kappa coefficient. Statistical significance was set at 0.05. Results: We included 221 patients (84.2% female, mean age of 45.4±13.5 years, mean disease duration of 15.4±9.5 years). In this preliminary study, the proposed cut-off values of SLE-DAS to define each disease activity category were: remission SLE-DAS≤2.08, LDA 2.08 7.64 for (Table 1). The overall accuracy of these SLE-DAS cut-off values to identify each disease activity state was 96.4%. The agreement between SLE-DAS and physician’s classification was very high (k=0.925, p According to the SLE-DAS cut-offs, 68.8% of the patients were in remission, 2.3% in LDA, 10.9% in mild disease activity and 18.1% in moderate/severe disease activity. Conclusion: The SLE-DAS has a high precision in identifying remission, LDA, and other disease activity states in SLE. These results suggest that the SLE-DAS is an accurate tool in defining achievable targets in SLE management. References: [1] Jesus D, Matos A, Henriques C, et al. Derivation and validation of the SLE Disease Activity Score (SLE-DAS): a new SLE continuous measure with high sensitivity for changes in disease activity. Ann Rheum Dis 2019, Epub ahead of print 9 January 2019. DOI: 10.1136/annrheumdis-2018-214502. Disclosure of Interests: None declared

Background Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are multi-systemic diseases associated with anti-neutrophil cytoplasmic antibodies (ANCA). Patients develop severe and irreversible damage, even in early stages of the disease, but data about prognostic factors are limited. Objectives To assess items and predictors of severe damage in a monocentric cohort of ANCA-associated vasculitis (AAV) patients, classified as GPA or MPA. Methods Clinical and serological data of patients, followed-up in a daily practice setting, were retrospectively revised. Severe damage, defined as a Vasculitis Damage Index (VDI) 3 5, was assessed after 12 months and at last examination (LE). The five most important items, predictors of severe damage and their increase rate were analysed at the beginning and at the last examination. Results 69 patients were included, 53 (76.8%) classified as GPA and 16 (23.2%) as MPA. According to EUVAS classification 7 (10.1%) were affected by limited disease, 14 (20.3%) by early systemic disease, 35 (50.7%) by generalized disease and 13 (18.9%) by severe disease. Patients were middle aged at diagnosis (54.6±15 years) with a diagnostic latency of 9.6±16.4 months, mostly caucasian (98.6%) and equally represented in sex (female 53.6%). Mean follow-up was 54.5±41.9 months. The number of flare/year at LE was significantly higher in anti-PR3 patients if compared to anti-MPO (1.0±1.1 vs 0.3±0.5, p=0.028). The VDI items most frequently observed are listed in Figure 1. We measured their frequencies and increase rate from diagnosis to the LE: arterial hypertension increased from 3 (4.7%) to 41 (65.1%) (+60.3% (IC 95% 48–71%), p 0.5 g 24 h from 1 (1.6%) to 13 (20.6%) (+19% (IC 11–31); p=0.0006). Severe damage, defined as VDI≥5, increased over time, being present in none patient at diagnosis and in 14 (25.9%) at last examination. At univariate analysis, number of flares/year (p=0.012), BVASv3 at diagnosis (p=0.03), steroid pulses therapy (p=0.04) and steroid cumulative dose (p=0.03) were identified as predictive factors of severe damage in the first year. Conversely, creatinine at LE (p=0.04), numbers of hospitalizations (p=0.009) and steroid cumulative dose at LE (p=0.47) were found to be predictors of long-term severe damage. Interestingly, ANCA pattern did not influence damage severity and only slightly differences in VDI items was noted between anti-PR3 and anti-MPO positive patients. Conclusions Our results highlights that knowing severe damage predictors is key in short and long term treat to target patient9s management. Disclosure of Interest None declared