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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 Patients with Systemic Lupus Erythematosus (SLE) are often treated with prolonged maintenance therapy with immunosuppressants (ISs) after remission achievement, with the aim of avoiding disease flares and subsequent organ damage. Data on the risk of flare after IS discontinuation and the effect of IS discontinuation on damage accrual are scanty. Objectives Our aims were to analyze damage progression in remitted SLE patients who did or did not discontinued ISs, to assess flare rate after IS withdrawal and to compare damage accrual in patients who did or did not flare after IS discontinuation. Methods We considered all SLE patients included in our lupus database, diagnosed between 1990 and 2018 (ACR criteria), treated with immunosuppressants over their disease course, who discontinued IS due to remission. IS discontinuation was defined as complete withdrawal of any immunosuppressive drug, and remission as clinical SLE Disease Activity Index (SLEDAI)-2K=0. Flares were defined according to SLEDAI Flare Index, and damage according to SLICC damage Index (SDI). Results Eligible patients ever treated with ISs were 319 out of 456 (69.9%) currently in follow-up. Remission lasting at least 6 months was achieved by 206 patients treated with IS (64.6%) (Table 1); among them 105 (51%) discontinued ISs during the follow-up. Mean±SD follow-up after IS withdrawal was 91±71 months (range 6-372). No difference in damage accrual between remitted patients who discontinued or did not discontinue ISs was observed at the end of follow-up, after adjusting for disease duration: median (range) SDI 1 (0-6) and 0 (0-4), respectively. Accordingly, the proportion of remitted patients who accrued damage during the follow-up was similar between those who did or did not discontinue ISs (55% vs. 48%). Among patients who discontinued ISs, 26 (24.7%) experienced a flare after a median (range) of 57 (6-264) months from IS discontinuation. Flares were severe in 50% of cases (Table 2). No difference in damage progression between patients who flared and did not flare after IS withdrawal was found at the end of follow-up: median (range) SDI 1 (0-5) and 1 (0-6), respectively. Moreover, the proportion of patients with damage accrual was similar among patients with and without flare after IS discontinuation (56% vs. 54%). Conclusion In our SLE cohort, the withdrawal of IS therapy in remitted patients did not seem to influence damage progression in the medium-term. Disclosure of Interests: 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: Remission is the most desirable target in the treatment systemic lupus erythematosus (SLE) however, a universally accepted definition of remission in SLE is still missing. Objectives: To test the contribution of the different items included into the currently used definitions of remission in SLE. Methods: We studied 646 Caucasian patients from a multicentre lupus cohort followed for at least 5-years: female 585 (90.6%), mean age at baseline 40.59±12.14 years, mean disease duration 9.18±6.86 years. Disease activity was assessed by clinical SLE Disease Activity Index 2000 (cSLEDAI) and SELENA-SLEDAI physician global assessment (PGA), and damage by Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI). To test the performance of the different items included in the definitions of SLE remission [1, 2] we identify 7 subtypes of remission: (1) PGA (0-3) Results: The number of patients achieving remission according to the different definitions is show in Figure 1. The proportion of patients who maintained prolonged remission (5-consecutive years) was: PGA When PGA Conclusion: The prevalence and extent of damage significantly decreased as the time spent in remission increased, irrespective of the remission subtype. The addition of PGA References [1] van Vollenhoven R, et al. 2014 [2] Zen M, et al. 2015 Disclosure of Interests: Francesca Saccon: None declared, Margherita Zen: None declared, Mariele Gatto: None declared, Domenico PE Margiotta: None declared, Fulvia Ceccarelli: None declared, Giulia Frontini: None declared, Gabriella Moroni: None declared, Alessandra Bortoluzzi: None declared, Marcello Govoni: None declared, Viola Signorini: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB, Francesca Dall’Ara: None declared, Angela Tincani Consultant for: UCB, Pfizer, Abbvie, BMS, Sanofi, Roche, GSK, AlphaSigma, Lillly, Jannsen, Cellgene, Novartis, Anna Chiara Frigo: None declared, Antonella Afeltra Grant/research support from: MSD, PFIZER, ABBVIE, ROCHE, UCB, Speakers bureau: MSD, PFIZER, BMS, ROCHE, SANOFI, fabrizio conti: None declared, Andrea Doria: None declared

Background The efficacy of tacrolimus (TAC) in lupus nephritis (LN) has been recently demonstrated in several randomised controlled trials in asian cohorts.1 Nevertheless, data on real-life experience and long-term safety of TAC are still lacking. Objectives To assess efficacy and safety of TAC in SLE patients with refractory LN in a multi-centric prospective cohort. Methods Adult SLE patients with refractory LN from 4 Italian centres were prospectively followed for at least 12 months. Clinical and serological variables and previous/concomitant medications were collected at baseline,3,6,12 months after starting TAC. Renal response was assessed by using EULAR/ERA-EDTA definitions.2 Adverse events (AEs) included non-infectious and infectious AEs; they were defined severe (SAE) when hospitalisation and/or death and/or life-threatening manifestations occurred. Data were analysed using SPSS (v.24.0). Results Thirteen SLE patients (8 females,5 males, mean age 33.7±10.2, mean disease duration 11.9±5.9) were enrolled (table 1). Concomitant medications included: prednisone(100%), mycophenolate mofetil(92.3%), hydroxychloroquine(53.8%), azathioprine(7.7%), belimumab(7.7%) and cyclosporine(7.7%). The average number of previous immunosuppressants taken by each patient was 3.15±1.90. Six-months renal response was achieved by 5/13 patients (38.5%): complete renal response (CRR) in 3 (60.0%) and partial renal response (PRR) in 2 patients (40.0%). Twelve-months renal response occurred in 4/13 patients (30.8%): CRR in 2/13 (50.0%) and PRR in 2/13 (50.0%). One patient achieved CRR at 6 and 12 months; one patient achieved PRR at 6 months and CRR at 12 months. Six patients (46.1%) discontinued TAC after an average follow up period of 10.2±7.8 months. Causes of discontinuation were: 3 non-infectious non-SAEs, 1 flare, 1 non-infectious SAE, and 1 non-response. Concerning AEs, no severe infections or deaths occurred. Conclusions Our preliminary data from clinical practice setting suggest that TAC could be a therapeutic rescue strategy for refractory LN. Further studies are needed to prove TAC efficacy in the long term. References [1] Mok CC, Ying KY, Yim CW, et al. Tacrolimus versus mycophenolate mofetil for induction therapy of lupus nephritis: a randomised controlled trial and long-term follow-up. Ann Rheum Dis2016;75:30e6. [2] Bertsias GK, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann Rheum Dis2012;0:1–12. Disclosure of Interest None declared

Background: SLEDAI is a widely used instrument to measure disease activity of systemic lupus erythematosus (SLE). However, it lacks sensitivity to discriminate improvement/worsening as it only scores items categorically and does not include several relevant lupus features, such as hemolytic anemia. Objectives: To derive and validate a SLE Disease Activity Score (SLE-DAS) with improved sensitivity to change, while maintaining the high specificity and simplicity of use of the SLEDAI. Methods: 324 patients fulfilling ACR’97 and/or SLICC’12 classification criteria for SLE and regularly followed at a tertiary care lupus clinic from January 2014 to December 2017 were included. At each outpatient visit, clinical and laboratory data were collected and disease activity (last 30 days) was scored with Physician Global Assessment (PGA) (0–3 scale) and SLEDAI-2K. To derive the SLE-DAS we analyzed data from the study visit with higher disease activity from each patient, applying multivariate linear regression analysis, with PGA as dependent variable/gold-standard. Independent variables tested in the models included items from SLEDAI-2K and continuous variables for swollen joint count, proteinuria, platelet and white blood cells counts. Some features absent from SLEDAI, such as hemolytic anemia, gastrointestinal and cardiopulmonary involvement were added to the model. To assess correlation validity we performed a Spearman’s correlation between the SLE-DAS, PGA and SLEDAI-2K at last follow-up visit. We tested performance of SLEDAI-2K (change ≥4) and SLE-DAS to discriminate a clinically meaningful worsening and improvement in SLE disease activity (change in PGA ≥0.3) using Receiver Operating Characteristic (ROC) curve analysis. We determined the best cut-offs values of SLE-DAS to detect changes in PGA ≥0.3 and calculated the sensitivity, specificity, positive and negative predictive values (PPV, NPV). Statistical significance was set at 0.05. Results: The final SLE-DAS model included 17 items. The SLE-DAS score at last follow-up visit presented high correlation with PGA (rho=0.975, p Conclusions: The SLE-DAS presents good construct validity and much higher discriminative power to detect changes in SLE disease activity as compared to SLEDAI-2K. External validation in another SLE cohort is underway. Disclosure of Interest: None declared

Background Prolonged treatment with immunosuppressants (IS) has been associated with long-term complications in Systemic Lupus Erythematosus (SLE); however, few data on IS discontinuation in remitted patients are available to date. Objectives We conducted an observational study to describe the proportion of SLE patients who discontinued IS and to assess the potential predictors of a subsequent flare. Methods We used data from Padua Cohort, which includes 454 SLE patients followed up from 1990 to 2017. Patients treated with IS over the disease course who discontinued IS and seen at least once in 2017 were studied. Reasons for discontinuation were: remission (defined by clinical SLE disease activity Index=0) or poor compliance/intolerance. Flares were defined according to SLEDAI Flare Index. Predictors of a subsequent flare were analysed by multivariate logistic regression analysis. Results Eligible patients who were ever treated with IS were 297. IS were discontinued in 106 patients (35.7%): mycophenolate (50, 47.2%), azathioprine (27, 25.5%), cyclophosphamide (11, 10.4%), methotrexate (10, 9.4%), cyclosporine (8, 7.5%). Mean ±SD follow-up duration after IS withdrawal was 82±64 months (range 6–320). 83 out of 106 patients (78.4%) discontinued IS due to remission (mean remission duration at IS discontinuation 39±28 months), and 23 (21.6%) due to poor compliance/intolerance. Among remitted patients, 18 (78.3%) experienced a flare after IS discontinuation (9/55 patients with nephritis, 5/10 with arthritis, 2/9 with skin involvement, 1/3 with neuroSLE, 1/4 with haematological involvement) after a mean of 65±52 months (range 6–180). Conversely, in patients with poor compliance/intolerance, 17 relapsed (73.9%) after a mean of 22±16 months. Flare-free 10 year-survival rate was higher in patients who discontinued IS due to remission than to poor compliance/intolerance (p In patients who discontinued IS due to remission, a shorter duration of remission at IS discontinuation was associated with disease relapse (p=0.006). Patients who were on IS due to nephritis had a lower risk of flare after IS discontinuation compared with patients with other manifestations (16.4% vs 32.1%, OR 0.58, 95% 0.32–0.98, p=0.049); patients with arthritis were those who were more likely to flare (OR 4.61, 95% CI 1.16–18.29, p=0.035). Positive anti-SSA/SSB (OR 0.45, 95% CI 0.26–0.78, p=0.012) and antimalarials intake after IS discontinuation (OR 0.22, 95% CI 0.07–0.73, p=0.015) were associated with a lower risk of flare. No clinical features over the disease course were associated with flare occurrence. At multivariate analysis, antimalarial use was the strongest protective factor against flares after IS discontinuation (OR 0.22, 95% CI 0.05–0.85, p=0.029). Conclusions In our cohort, one third of patients treated with IS discontinued the drug during the follow-up, in most cases due to a prolonged remission. Patients who discontinued IS due to remission had a higher free-survival rate than those who discontinued these drugs due to poor compliance/intolerance. The use of antimalarials after IS discontinuation was independently associated with a significant decrease in the risk of flare. IS discontinuation in patients with arthritis requires particular caution. Disclosure of Interest None declared

Background The treatment of neuropsychiatric systemic lupus erythematosus (NPSLE) is extremely challenging and only a few clinical trials have been performed to establish optimal management. Objectives To describe the therapeutic approach and the short-term outcome of a multi-centre cohort of patients with NPSLE, enrolled at the time of the first NP event. Methods This is a retrospective cohort study. All NP events were defined according to American College of Rheumatology (ACR) case definition and divided into 3 clusters: central/diffuse (C/D), central/focal (C/F) and peripheral (P). A validated attribution algorithm was used to determine the attribution of all NP events. Demographic variables, global SLE disease activity Index 2000 (SLEDAI-2K), cumulative organ damage (SLICC/ACR Damage Index (SDI)) and treatment adopted for NP manifestations were collected. The clinical outcome of all NP events was determined by a physician-completed seven-point Likert scale (1=patient demise, 2=much worse, 3=worse, 4=no change, 5=improved, 6=much improved, 7=resolved). The relationship between the variables of interest and the outcome was analysed by crude and adjusted logistic models and reported as Odds Ratio (OR) and 95% confidence intervals (95% CI). Results 461 SLE patients with at least one NP event were included. 91.8% of patients were female, mean (SD) age 35.4 (13.6) years. 19.7% (91) of events were observed at diagnosis of SLE, 13.4% (62) before and 66.8% (308) after the diagnosis. 111 events (24.1%) were C/F, 286 (62%) C/D and 64 (13.9%) P. 198 (42.95%) of all NP events were attributed to SLE. The overall probability of immunosuppressive therapy was 28.4% (95% CI 24.3–32.8), 38.7% (95% CI 29.6–48.5) in C/F, 21.3% (95%CI 16.7–26.5) in C/D and 42.2% (95%CI 16.7–26.5) in P manifestations. The probability of immunosuppressive therapy was 47.9% (95% CI 40.8–55.2) in attributed events. The one-year outcome was available in 355 patients. Physician assessment indicated resolution (76 patients) or improvement (150 patients) in 49% (226/461) of cases. The crude and adjusted OR of attributed NP events and immunosuppressants on a favourable outcome is illustrated in Figure 1. The multivariable logistic regression analysis was done adjusting for age at diagnosis of SLE [OR 0.96, 0.94–0.98] p=0.001, female gender [OR 0.97, 0.33–2.7] p=0.959, SDI [0.85, 0.68–1.08] p=0.202, SLEDAI-2K [1.06, 1.01–1.11] p=0.008 and type of event (F/C [REF], C/D [0.37, 0.16–0.83] p=0.016, P [0.54, 0.21–1.42] p=0.215). Conclusions In our study, the therapeutic immunosuppressive approach was mostly used in attributed, C/F and P manifestations. In patients treated with immunosuppressants, the favourable outcome was lower in C/D phenotype. Disclosure of Interest None declared

Objectives To evaluate survival of prednisone (PDN) – free remission in systemic lupus erythematosus (SLE) patients and to investigate the potential predictors of disease flares. Methods Inclusion criteria were: (1) Diagnosis of SLE according to American College of Rheumatology (ACR) Classification Criteria of SLE; (2) Caucasian ethnicity; (3) Clinical remission (clinical SLEDAI-2K=0) at the time of PDN-withdrawal; (4) Stop of PDN treatment between 2010 and 2016; (5) At least two visits per year between January 2010 and April 2016. Disease activity was assessed according to SLE Disease Activity Index-2000 (SLEDAI-2K). Damage was measured by the SLICC/American College of Rheumatology Damage Index (SDI). Flares were defined according to Safety of Estrogens in Lupus Erythematosus National Assessment (SELENA)-SLEDAI criteria. We evaluated whether gender, age, age at PDN-stop, disease duration, duration of corticosteroid therapy, duration of remission before PDN withdrawal, time to flare, SLICC/American College of Rheumatology Damage Index (SDI) – score >3, positive anti-dsDNA antibodies (abs) and-or low C3/C4, type of SLE-involvement and concomitant immunosuppressive treatment could be predictors of flare. Multivariate logistic regression analysis was run to investigate the predictors of flare. Covariates included in the analysis were all variables reaching p Results Among 400 patients evaluated, 104 (26%) fulfilled inclusion criteria. Baseline characteristics are reported in table 1. Twenty-two (21.2%) patients flared. Mean time to flare was 19.91±13.14 months. Types of flare were 7 renal, 7 articular, 4 cutaneous, 2 haematological, 1 serositic and 1 neurological. Variables included in the multivariate logistic regression analysis were: positive anti-dsDNA abs and-or low C3/C4, skin, articular and haematological involvement. Skin involvement resulted predictive of flare (OR 3.07, 95% CI 1.11–8.53, P 0.031) as reported in table 2. Conclusions In SLE patients who stopped corticosteroid therapy, previous skin involvement resulted to be a predictor of disease flare. Disclosure of Interest 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