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Background: In the last few decades many studies have investigated the role of major salivary glands ultrasound (SGUS) in the diagnostic work-up of patients with suspected primary Sjogren’s syndrome (pSS). Recent development of ultra-high-resolution ultrasound systems (UHFUS), with frequencies as high as 70 MHz and capability resolution as fine as 30 μm, has permit new diagnostic applications to a variety of superficial targets including labial salivary glands (LSGs). To date, however, no information are available regarding the use of UHFUS for the study of LSGs in humans. Objectives: To evaluate the feasibility and the diagnostic accuracy of UHFUS of LSGs in patients with suspected SS and to assess the correlations between LSG histopathology, UHFUS and SGUS. Methods: Consecutive patients with clinically suspected pSS were included in this study. All patients underwent a complete diagnostic work-up including conventional SGUS and LSG biopsy. The same expert pathologist calculated for all the samples the focus score (FS), number of foci and evaluated the presence of ectopic germinal centers (GCs). UHFUS of LSG was performed by specialized radiologists scanning first the central compartment of the inferior lip, and then both peripheral compartments. The following parameters were evaluated: distribution of the glands, parenchymal inhomogeneity (score 0-3, from normal to evident), fibrosis and eco color-Doppler vascular pattern and grade of vascular intensity. Results: We included 32 patients with suspected pSS. At the end of the work-up, pSS diagnosis was confirmed in 12/32 (37.5%) cases. No differences between pSS patients and no-SS sicca controls were observed in UHFUS findings related to LSG distribution and eco color-Doppler vascular parameters. By contrast, pSS patients presented statistically significant differences in peripheral UHFUS inhomogeneity (p=0.006), and a higher degree of fibrosis (p=0.01). Considering a score ≥2 in parenchymal inhomogeneity as suggestive for pSS, UHFUS appeared less specific than conventional SGUS (UHFUS Sp=70% vs SGUS Sp=95%) but more sensitive (UHFUS Se=83.3% vs SGUS Se = 58.3%). In addition, when we investigated the relationship between UHFUS, SGUS and LSG histopathology we found that the correlation coefficients between UHFUS inhomogeneity and LSG FS (UHFUS r=0.706** vs SGUS r=0.393*), number of foci (UHFUS r=0.712** vs SGUS r=0.445*), and number of ectopic GCs (UHFUS r=0.525** vs SGUS r=0.141), were significantly higher than those observed with conventional SGUS. Conclusion: the application of UHFUS to the study of LSG in pSS appeared feasible and sensitive. Because of the anatomical details obtained with this technique and its stronger correlation with LSG histopathology, UHFUS might offer unique advantages over the existing major salivary gland imaging modalities in pSS assessment. Disclosure of Interests: Chiara Baldini: None declared, Francesco Ferro: None declared, Veronica Iodice: None declared, Rossana Izzetti: None declared, Valentina Donati: None declared, Silvia Fonzetti: None declared, Teresa Oranges: None declared, Valentina Dini: None declared, Dania Cioni: None declared, Nicoletta Luciano: None declared, Rosa Maria Bruno: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB, Saverio Vitali: None declared, Davide Caramella: None declared

It is generally acknowledged that, in SLE, disease activity is associated with poor prognosis, high glucocorticoid use, damage development and increased mortality; therefore reaching low level of disease activity or remission are main treatment targets for SLE. Definitions for disease remission and low disease activity are being proposed and validated as meaningful targets to be pursued in SLE management. In detail an international task force has recently developed a definition of remission in SLE (DORIS) taking in consideration four different domains: clinical activity, serological activity, treatment and duration. This approach led to the development of different levels of remission i.e. clinical remission on/off treatment and complete remission on/off treatment. Recently data from different cohorts have been published, showing that remission is an achievable target in SLE. Achieving remission appears associated with reduced damage accrual, however persistence in remission is rare being maintained in an average of 7% of patients over 5 years. In addition, the more stringent is the definition, the more difficult is to achieve remission and a longer time to remission is observed in patients with high activity, high therapy, hematological activity, African-American ethnicity. Low disease activity may represent another target in SLE treatment and a definition of (lupus low disease activity state (LLDAS) has been developed by Franklyn et al. LLDAS is defined based on three domains which are SLEDAI-2k 4, physician global assessment (PGA) 1, absence of new manifestations and stable and well tolerated treatment with a prednisolone (or equivalent) dose of 7.5 mg/day. Interestingly, LLDAS is achieved by a high percentage of patients over follow up, ranging between 33 and 88% in different cohorts and it is maintained over follow up in up to 50% of patients for 50% of follow up time. Predictive factors for LLDAS attainment are shorter disease duration, lower disease activity score, lower mean PGA, lower mean SLEDAI, older age; while persistent LLDAS is less frequent in patients with vasculitis, neurological, renal, cardiopulmonary and mucocutaneous manifestations. Achievement and persistence of LLDAS are associated with lower prednisone dose during follow up, reduction of disease flares, lower damage accrual, better quality of life. In conclusion, definitions of remission and low disease activity in SLE have been proposed and validated against outcomes such as glucorticoids usage, damage accrual, quality of life. Both targets are associated with improved outcomes, however at present persistence in remission is not common. The achievement of LLDAS is not rare, persistence in LLDAS is achievable. References: [1] van Vollenhoven RF, Mosca M, Bertsias G, et al. Treat-to-target in systemic lupus erythematosus: recommendations from an international task force. Ann Rheum Dis 2014;73:958-67. [2] van Vollenhoven R, Voskuyl A, Bertsias G, et al. A framework for remission in SLE: consensus findings from a large international task force on definitions of remission in SLE (DORIS). Ann Rheum Dis 2017;76:554-561. [3] Franklyn K, Lau CS, Navarra SV, et al. Asia-Pacific Lupus Collaboration. Definition and initial validation of a Lupus Low Disease Activity State (LLDAS). Ann Rheum Dis 2016;75:1615-21. [4] Petri M, Magder LS. Comparison of Remission and Lupus Low Disease Activity State in Damage Prevention in a United States Systemic Lupus Erythematosus Cohort. Arthritis Rheumatol 2018. [5] . Tsang-A-Sjoe MW, Bultink IE, Heslinga M, et al. Both prolonged remission and Lupus Low Disease Activity State are associated with reduced damage accrual in systemic lupus erythematosus. Rheumatology 2017;56:121-28. [6] Zen M, Iaccarino L, Gatto M, et al. Lupus low disease activity state is associated with a decrease in damage progression in Caucasian patients with SLE, but overlaps with remission. Ann Rheum Dis 2018;77:104-10. [7] Zen M, Iaccarino L, Gatto M, et al. The effect of different durations of remission on damage accrual: results from a prospective monocentric cohort of Caucasian patients. Ann Rheum Dis 2017;76:562-65. [8] Tselios K, Gladman DD, Touma Z, et al. Clinical remission and low disease activity have comparable outcomes over 10 years in systemic lupus erythematosus. Arthritis Care Res 2018. Disclosure of Interests: Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB

Background The European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ERN ReCONNET) is a virtual Network that aims to improve and standardize the quality of care offered to rCTDs patients in Europe, empower patients, share knowledge and expertise, enhance research, support efficient use of resources. ERN ReCONNET covers 10 rCTDs: APS, UCTD, Idiopathic Inflammatory myopathies, IgG4, MCTD, Systemic sclerosis, Relapsing Polychondritis, SLE, Sjogren and EDS. Objectives Identification of unmet needs in diagnosis, monitoring and management of rCTDs after literature review of existing clinical practice guidelines (CPGs). Methods After the review of existing CPGs, the most relevant unmet needs, both for clinicians and patients, were identified by discussion among the members of the network. Results A considerable number of unmet needs were identified. In particular, the lack of shared classification criteria, of evidence-based CPGs, validated tools for the assessment of treatment response and disease activity/damage are the major unmet needs especially for the rarest rCTDs. Transversal topics for all rCTDs that need to be addressed are the scarcity of EU/international randomised controlled trials, the identification of patient-reported outcomes and non-adherence to treatment, quality of life indicators, the need to develop composite disease activity scores for all rCTDs and specific (inter)national web-based registries. Patients highlighted the need of a more holistic approach to rCTDs, demanding more attention to pain, fatigue and psychological aspects related to the diseases and the promotion of an early diagnosis. Conclusion The identification of rCTDs unmet needs provided a very useful picture on future actions to be undertaken in order to provide a better care to patients. Many activities are ongoing towards these goals in ERN ReCONNET. These will be possible as a result of a EU collaboration among rCTDs stakeholders, the main added value of ERN ReCONNET. Acknowledgement Thanks to all ERN ReCONNET Steering Committe, Healthcare Providers, ePAGs and Team for huge support. Disclosure of Interests Diana Marinello: None declared, Simone Ticciati: None declared, Rosaria Talarico: None declared, Tobias Alexander: None declared, Laurent Arnaud Consultant for: Alexion, Amgen, AstraZeneca, GSK, Janssen-Cilag, LFB, Lilly, Menarini France, Novartis, Pfizer, Roche-Chugai, and UCB., Paid instructor for: Alexion, Amgen, AstraZeneca, GSK, Janssen-Cilag, LFB, Lilly, Menarini France, Novartis, Pfizer, Roche-Chugai, and UCB., Speakers bureau: Alexion, Amgen, AstraZeneca, GSK, Janssen-Cilag, LFB, Lilly, Menarini France, Novartis, Pfizer, Roche-Chugai, and UCB., Lorenzo Cavagna: None declared, Lorenzo Beretta: None declared, Benjamin Chaigne: None declared, Alain Cornet Grant/research support from: No direct financial grants from Pharmaceutical companies, but some grants accrued LUPUS EUROPE does, in the form of Grants or payment for attending advisory boards or providing patient views, Speakers bureau: I have been involved in GSK panels to provide patient perspective. However, the minor service fee obtained accrued to LUPUS EUROPE ratyher than the company, Nathalie Costedoat-Chalumeau: None declared, Rebecca Fischer-Betz Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Charissa Hermine Frank: None declared, Ilaria Galetti: None declared, Jurgen Grunert: None declared, vera guimaraes: None declared, Luca Iaccarino: None declared, Maarten Limper Consultant for: GSK, Roche and Thermofisher, Speakers bureau: GSK and Roche, Fransiska Malfait: None declared, Xavier Mariette Grant/research support from: Servier, Consultant for: AstraZeneca, Bristol-Myers Squibb, GlaxoSmithKline, Janssen, Pfizer, UCB Pharma, lisa matthews: None declared, Marco Matucci-Cerinic Grant/research support from: Actelion, MSD, Pfizer, BMS, Chemomab, Sanipedia, Speakers bureau: Actelion, BMS; MSD, Janssen, alain meyer: None declared, Simona Rednic: None declared, Vasco Romao: None declared, Carlo Alberto Scire: None declared, Vanessa Smith: None declared, Alberto Sulli: None declared, Farah Tamirou: None declared, Anna Viola Taulaigo: None declared, Angela Tincani Consultant for: UCB, Pfizer, Abbvie, BMS, Sanofi, Roche, GSK, AlphaSigma, Lillly, Jannsen, Cellgene, Novartis, Ana Vieira: None declared, Maurizio Cutolo: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB

Background Detailed analysis of hematological manifestations are limited and their clinical impact on disease remain obscure. Objectives To scrutinize factors associated with different hematological abnormalities (HA) in SLE patients and their impact on infections, bleeding and damage accrual. Methods A dataset (GIPTSLE) originated from SLE patients in Europe was studied. The dataset consisted of six monthly visits of each patients for at least 2 years. Results were compared with another well-established SLE cohort from Sweden (Swedish SLE network). Patients in both cohorts fulfilled the ACR1982 SLE criteria. Variables collected at each visit included CBC, presence or absence of haemolytic anemia, all domains of SLEDAI-2K, medications, infection or bleeding episodes, Systemic Lupus International Collaborating Clinics/ACR damage index (SDI). HA occurred at the beginning or during follow-up were defined according to the ACR criteria. HA was only considered in patients with no prior use of immunsuppressives or biologic agents at least 3 months before the first HA events. The Swedish confirmation cohort included all the variables of interest. We excluded all haematological domains from SLEDAI-2K (aSLEDAI) for the analysis. Based on six monthly change in SDI, each visit was labelled either “damage transition”or “non-damage transition”. Generalized estimating equations (GEE), multiple logistic regression, Chi-square test and independent samples t test were used where it was appropriate. Results Of the 1430 visits of 286 patients analysed from the first cohort (89.5% female, 95.8% Caucasian, 64.7% dsDNA positive) 60% had at least one HA. At the enrolment, the median (range) disease duration was 8 yrs (0-38), aSLEDAI-2K was 6 (0-25) and SDI was 0(0-6). Upon GEE analysis, lymphopenia was significant for SDI (OR=2.5, 1.0-5.9).Upon GEE analysis after further adjusting prednisolone exposure, HA found be significant for SDI was thrombocytopenia (OR=3.6, 1.04-12.1) but no significant evidence for the dependence of this relationship on time. The effect of lymphopenia was attenuated. HR for bleeding was significant when platelets were below 50K (HR=2.6, 1.15-6.1) Of the 1395 patients analysed from the second set (86.8%female, 93.0% Caucasian, 61.6 dsDNA positive), 63.4% had HA. At the last visit, the median (range) disease duration was 14yrs (1-65) and SDI was 1(0-14). Upon multiple regression analysis after adjusting age, sex and prednisolone exposure both thrombocytopenia (OR=2.2, 1.3-3.6) and lymphopenia (OR=1.8, 1.2-2.6) had significant effect on SDI. HA at the time of diagnosis or occurring at any time during disease course did not contribute to mortality. Conclusion Lymhopenia contributes independently damage accrual in European background. Lymphopenia and thrombocytopenia at early disease appear to be independent risk factors for subsequent damage. References [1] Seminar in Arthritis and Rheumatism 45(2016):675-83 Disclosure of Interests Sule Yavuz: None declared, Dondu Cansu: None declared, Dionisis Nikolopoulos: None declared, Francesca Crisafulli: None declared, Ana M Arantunes: None declared, Christina Adamichou: None declared, Sara Reid: None declared, Chiara Satgnaro: None declared, Laura Andreoli: None declared, Angela Tincani Consultant for: UCB, Pfizer, Abbvie, BMS, Sanofi, Roche, GSK, AlphaSigma, Lillly, Jannsen, Cellgene, Novartis, Maria Moraes-Fontes: None declared, George Bertsias: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB, Dag Leonard: None declared, Antonis Fanouriakis Paid instructor for: Amgen, GSK, Speakers bureau: Abbvie, Enorasis, Genesis Pharma, Lars Ronnblom: None declared, SWEDISHSLE NETWORK: None declared

Background: Culminating evidence over the past decade regarding management of systemic lupus erythematosus (SLE) called for an update of the 2008 EULAR recommendations for the treatment of the disease. Objectives: Systematic review of the literature (SLR) to inform the 2019 EULAR recommendations for the management of SLE. Methods: SLR of Pubmed from 01/2007 to 12/2017 for questions (selected through Delphi excercise) regarding: i) efficacy/safety of different drugs used in SLE, ii) treatment of specific manifestations, iii) monitoring and treatment goals and iv) comorbidities and adjunct therapy. Evidence was categorised based on design and validity of available studies [Oxford Centre for Evidence-Based Medicine 2011 Levels of Evidence (LoE)] and strength of statements was graded [Grading of recommendation (GoR), assessment, development and evaluations, GRADE)]. Results: Main topics supported by strong evidence base included: Association of hydroxychloroquine (HCQ) use with favourable outcomes (LoE 1b, GoR A), belimumab for extrarenal disease (LoE 1a, GoR A), efficacy of antimalarials in skin disease (LoE 1a, GoR A), mycophenolate mofetil (MMF) for induction and maintenance therapy of lupus nephritis (LN) and cyclophosphamide (CYC) in severe LN (LoE 1b, GoR A). Weak evidence supported the value of repeat kidney biopsy in refractory LN (LoE 4, GoR C), all second-line agents for skin disease (LoE 4, GoR C) and efficacy of most first and second-line treatments for thrombocytopenia (LoE 4, GoR C). Moderate LoE was found for all other questions (Table). Conclusion: A SLR for the treatment of SLE found the highest LoE for benefits of HCQ, efficacy of belimumab for extrarenal disease and MMF and IV-CYC in LN. Acknowledgement: European League Against Rheumatism Disclosure of Interests: Antonis Fanouriakis Paid instructor for: Amgen, GSK, Speakers bureau: Abbvie, Enorasis, Genesis Pharma, Myrto Kostopoulou: None declared, Alessia Alunno: None declared, Martin Aringer Grant/research support from: Roche, Consultant for: AstraZeneca and Eli Lilly, Ingeborg Bajema Consultant for: GSK, John N. Boletis: None declared, Ricard Cervera: None declared, Andrea Doria: None declared, Caroline Gordon Grant/research support from: Sandwell and West Birmingham Hospitals NHS Trust have received funding from UCB to support research work done by my research group that was unrelated to any pharmaceutical product or clinical trial., Consultant for: I have provided consultancy advice and taken part in scientific advisory boards on the design and analysis of clinical trials and the management of lupus for GSK, EMD Serono and UCB. I have taken part in adjudication and safety monitoring committees for BMS., Speakers bureau: I have been paid by UCB for speaking at meetings., Marcello Govoni Paid instructor for: Pfizer, Roche, Speakers bureau: Pfizer, Abbvie, MSD, Roche, Eli-Lilly, Celgene, Sanofi, Janssen, Frederic Houssiau: None declared, David Jayne Grant/research support from: David Jayne has received research grants from Chemocentryx, GSK, Roche/Genentech and Sanofi-Genzyme. He has received consultancy fees from Astra-Zeneca, Boehringer-Ingelheim, Chemocentryx, Chugai, GSK, Infla-RX, Insmed and Takeda, Marios Kouloumas: None declared, Annegret Kuhn Grant/research support from: Biogen, Galderma, GlaxoSmithKline, LeoPharma, Speakers bureau: La Roche Posay, Janni Lisander Larsen: None declared, Kirsten Lerstrom: None declared, Gabriela Moroni: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB, Matthias Schneider Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Speakers bureau: Chugai, Josef S. Smolen Grant/research support from: AbbVie, Eli Lilly, Janssen, MSD, Pfizer Inc, Roche, Consultant for: AbbVie, Amgen, AstraZeneca, Astro, Celgene, Celtrion, Eli Lilly, GlaxoSmithKline, ILTOO, Janssen, Medimmune, MSD, Novartis-Sandoz, Pfizer Inc, Roche, Samsung, Sanofi, UCB, Speakers bureau: AbbVie, Amgen, AstraZeneca, Astro, Celgene, Celtrion, Eli Lilly, GlaxoSmithKline, ILTOO, Janssen, Medimmune, MSD, Novartis-Sandoz, Pfizer Inc, Roche, Samsung, Sanofi, UCB, Elisabet Svenungsson: None declared, Vladimir Tesar: None declared, Angela Tincani Consultant for: UCB, Pfizer, Abbvie, BMS, Sanofi, Roche, GSK, AlphaSigma, Lillly, Jannsen, Cellgene, Novartis, Anne Troldborg: None declared, Ronald van Vollenhoven Grant/research support from: AbbVie, Amgen, Bristol-Myers Squibb, GlaxoSmithKline, Pfizer, Roche, and UCB, Consultant for: AbbVie, AstraZeneca, Biotest, Bristol-Myers Squibb, Celgene, Crescendo, GlaxoSmithKline, Janssen, Lilly, Merck, Novartis, Pfizer, Roche, UCB, and Vertex., Speakers bureau: AbbVie, AstraZeneca, Biotest, Bristol-Myers Squibb, Celgene, Crescendo, GlaxoSmithKline, Janssen, Lilly, Merck, Novartis, Pfizer, Roche, UCB, and Vertex., Jorg Wenzel Grant/research support from: GSK, Incyte, Consultant for: Biogen, Leo, Paid instructor for: Novartis, George Bertsias: None declared, Dimitrios Boumpas: None declared

Background physician-based assessment of Systemic Lupus Erythematosus (SLE) may not be able to capture the real disease impact on patients’ life. In the literature, the impact of disease damage on patients’ quality of life (QoL) is controversial. Objectives: Objective of our study was to investigate the role of organ damage in determining patient perception of SLE burden. Methods: this is a cross-sectional study that enrolls patients with a diagnosis of SLE (ACR 1997 criteria). For each patient, demographics, comorbidities, treatment, clinical and laboratory data were collected. Disease damage was evaluated with the SLICC-Damage Index (SDI) and a score >2 was defined as “severe damage”. The BILD (Brief Index of Lupus Damage) was used for patient self-evaluation of organ damage. Finally, the Lupus Impact Tracker (LIT) questionnaire was used to assess patient perception of SLE burden. Results: we included 246 adult SLE patients (94.7% Caucasian, 93.1% female, mean age 45.31±13.2 years, mean disease duration 14.3±9.8 years). As for cumulative organ involvement in our cohort, the most prevalent was articular involvement (67.5%), followed by cutaneous (54.1%), hematological (51.2%), renal (43.9%) and serositis (17.9%); 11.8% had a history of NPSLE. Among comorbidities, 10.9% of patients had a concomitant fibromyalgia. 48.8% of patients was presenting at least one organ damage; among those patients, the median SDI was 2 (IQR 1-3); 16.3% of patients had a “severe damage” and among them median SDI was 4 (IQR 3-6). The most frequent items of organ damage in our cohort were: cataract (19.9%), deforming or erosive arthritis (10.2%) and osteoporosis with fracture (8.5%). Moreover, a significant number of patients (18.3%) met the criteria for neuropsychiatric damage, mainly cerebrovascular accidents (7.3%), seizures (5.3%) and cognitive impairment (4.1%). Finally, a not negligible number of patients had premature gonadal failure (4.5%) and malignancy (6.5%). As far as patients’ perception is concerned, the median LIT score in the cohort was 22,5 (IQR 7.5-40) and median BILD was 1 (IQR 0-2). In a multiple linear regression analysis, we found a direct positive correlation between the SDI score and age at enrollment and disease duration (p Among the different types of organ damage, we found that cognitive impairment, cerebrovascular accidents and premature gonadal failure mainly contribute to determine SLE burden. In fact, they were significantly associated with higher LIT scores (p Conversely, SDI score was not related with health-related quality of life and fatigue as measured by SF-36 and FACIT respectively, neither with fibromyalgia. Conclusion: disease damage seems to have a role in determining patient perception of SLE burden, mainly affecting patients’ ability to plan the future and to fulfill daily activities and family responsibilities. In particular, neuropsychiatric damage exerts the greatest influence on patient perception of SLE impact. References: [1] Yazdany J. et al, Arthritis Care Res (Hoboken). 2011 Aug;63(8):1170-7 Disclosure of Interests: Elena Elefante: None declared, Chiara Tani: None declared, Francesco Ferro: None declared, Chiara Stagnaro: None declared, Alice Parma: None declared, Linda Carli: None declared, Viola Signorini: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB

Background The most effective drug for the maintenance therapy of severe forms of lupus nephritis (LN) is still a matter of debate. Objectives To compare efficacy and safety of cyclosporine (CYA) with mycophenolate mofetil (MMF) and with azathioprine (AZA) in the long-term maintenance therapy of LN. Methods Ninety-six patients (pts) (93 females, mean age 31.98±12.78 years) with SLE and biopsy proven LN (16 pts:class III; 64 pts:class IV; 16 pts:class V ISN/RPS). Fifty-six pts entered this study at diagnosis of LN and 40 during a course of a LN flare. Twenty-five pts (30%) had Glomerular Filtration Rate (eGFR) 60ml/min and proteinuria 60ml/min and proteinuria >0.5/die, no response: eGFR Results At the beginning of maintenance therapy, the mean serum creatinine and eGFR were similar in the 3 groups (0.92±0.26mg/dl, eGFR 109,9±49,5ml/min in CYA, 0.86±0.4mg/dl, eGFR 119±44,6 in MMF, 0.85±0.3mg/dl, eGFR 106,6±43,9ml/min in AZA). Proteinuria was higher in CYA group (CYA:2.03±1.7g/day; MMF:0.77±0.8g/day; AZA:1.2±1.1g/day). At the beginning of maintenance therapy, complete, partial and no response were 26.6%, 60%, and 13.4% in CYA, 53.1%, 43.8% and 3.1% in MMF and 38.2%, 58.8% and 3% in AZA group (Fig 1). At 1 year, after 6 months of maintenance therapy, in CYA group the percentage of pts in complete remission increased to 73% (vs 65.6% in MMF and 40% in AZA), at 5 years it was 80% (vs 83% in AZA and in MMF) and 88% at 10 years vs 70% in MMF and 68% in AZA (Fig 2,3,4). The percentage of non-responsive pts was stable from 1 to 10 years in the CYA group (around 13%), it slightly increased in MMF group (from 3.1 to 13,5%) and in AZA group (from 15 to 24%). During the study, SLE flares occurred in 30% of CYA group, 41% in MMF and 32% of the AZA. The average time from the beginning of the study and the first flare was 3.95±2.76years in CYA, 3.62±1.60 in MMF and 5.9±2.37 in AZA. No side effects were reported in 90% of pts treated with CYA, in 81.3% with MMF and in 85.3% with AZA group. Conclusion This is the first study comparing these 3 drugs as maintenance therapy in the long term. After 10 years of observation, CYA, AZA and MMF have proven to be effective in consolidating and maintaining the remission of LN. Of interest are the results achieved in the CYA group. Despite worse clinical conditions at the beginning of maintenance therapy, CYA allowed a rapid achievement of LN remission in the great majority of pts compared to AZA and MMF. Remission persisted over 10 years of observation. The number and type of flares and of side effects were not different between groups. Reference [1] Moroni G, et al. 2006 Disclosure of Interests Lorenza Maria Argolini: None declared, Elena Elefante: None declared, Francesca Saccon: None declared, Valentina Binda: None declared, Maria Gerosa: None declared, Luigi Sinigaglia Speakers bureau: Yes, I,ve been invited speaker by Amgen, Ely Lilly, UCB, Abbvie, Roche and BMS., Piergiorgio Messa: None declared, Andrea Doria: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB, Gabriella Moroni: None declared

Background: Undifferentiated connective tissue disease (UCTD) is a group of systemic autoimmune conditions which do not fulfil the criteria for a definite connective tissue disease (CTD). The interaction between pregnancy and UCTD is still poorly studied and scarcely recognized. Objectives: The purpose of this study was to evaluate the risk of disease flares or differentiation into a well-defined CTD and also the risk of adverse outcome in pregnancy in patients with UCTD. Methods: A total of 100 pregnancies in 81 UCTD followed at a single center were included in this analysis. From the time the pregnancy was detected, each patient was evaluated every 4 – 6 weeks and the diagnosis was re-evaluated at each observation; the follow-up was considered completed after the last visit during puerperium. At each visit, patients were assessed by a rheumatologist and by a gynecologist. Results: Eleven pregnancies (11%) ended with abortion in the first trimester and the remaining 89 (89%) ended with a live birth. Twelve patients (12%) flared during pregnancy or puerperium and three (3%) had major flares and evolved to systemic lupus erythematosus (SLE) with renal involvement. Obstetric complications were observed in twenty-nine out of the 89 successful pregnancy (33%), including two cases (2%) of preeclampsia. We found a significant association between anti-dsDNA antibodies positivity at baseline and disease flare (p Conclusion: The impact of pregnancy appears to be less serious in UCTD than in other CTDs, however disease flares and obstetric complications can represent a clinical challenge; disease clinical and serological activity seem important determinants of pregnancy outcomes. Thus, a pre-pregnancy counselling and planning as well as a close monitoring during pregnancy is warranted. Disclosure of Interests: Dina Zucchi: None declared, Chiara Tani: None declared, Elena Elefante: None declared, Linda Carli: None declared, Alice Parma: None declared, Francesca Anna Letizia Strigini: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB

Background: Behcet’s Syndrome (BS) is globally characterized by a variable spectrum of disease profile: while prevalent muco-cutaneous lesions and arthritis represent the only clinical features in patients with a benign disease subset, there are other patients who develop potentially sight or life-threatening manifestations, due to ocular, neurological or major vascular involvement. Beside the organ involvement, demographic factors could considerably influence the long-term and short-term outcomes of BD Objectives: The primary aim of the study was to evaluate disease activity in a cohort of BS patients consecutively followed in a BS clinic of a tertiary centre; the secondary aims were to identify potential predictive factors of disease activity and to compare disease activity with quality of life. Methods: One-hundred and thirty patients (71 males and 59 females; mean age 42±8 years, mean disease duration 11±4) with a diagnosis of BS according to the ISG criteria were studied. Disease activity has been evaluated by BDCAF and quality of life by the Short form (SF) 36. Patients were also categorized in major or minor involvement of BS according or not to the presence of ocular, neurological and vascular involvement in the course of disease Predictors of long-term outcome were identified by univariate analysis using the log-rank test and by multivariate analysis using Cox proportional hazards regression models. Results: At time of the evaluation, 39% of BS patients presented an active disease. Thirty-six patients presented muco-cutaneous involvement, 21 ocular disease, 10 joint involvement, 6 neurological impairment and 4 gastro-enteric involvement. Sixty-two percent of patients presented in the course of the disease a severe BS involvement and, among these, the majority was represented by males HLAB51 positive patients. The major limitations of quality of life related to disease activity were represented by vitality, physical functioning, bodily pain, general health perception. Conclusion: Younger men patients affected by BS are more suitable to have a more severe disease, due to an increased frequency both of morbidity and mortality, secondary to ocular, vascular and neurological involvement. The variable prognosis associated to different gender or age, may represent an essential and useful element to tailor the management not only to the type and severity of symptoms, but also to the epidemiological profile of BD patients. Disclosure of interests: Rosaria Talarico: None declared, alice Parma: None declared, Eralda Zera: None declared, Emanuele Calabresi: None declared, Chiara Tani: None declared, Chiara Baldini: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB

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 Systemic sclerosis (SSc) is an autoimmune chronic disease characterized by prominent vascular involvement. Intravenous iloprost (IV ILO), according to the recently updated EULAR recommendations, is indicated for Ssc Raynaud’s phenomenon (RP) after failure of oral therapy. Moreover, IV ILO could be useful in DU healing and may represent a potential disease modifying medication. However, there are no uniform data regarding type of regimen (dosage, duration and frequency) and infusion modality. Objectives The purpose of this study was to compare effectiveness (in terms of Dus) and direct costs, of two regimens of IV ILO infusion in the same cohort of consecutive SSc subjects. Methods Protocol A (A): the patient was admitted and 100 mcg of ILO was diluted in 500 ml of normal saline or 5% dextrose solution and infused continually at the maximum tolerated dose until exhaustion. Protocol B (B): the patient was followed as outpatient at infusion clinic and 50 mcg of ILO was diluted in 250 of normal saline or 5% dextrose and infused at a dose range from 0.5 to 1.5 ng/Kg/min. The dose was escalated at 30 minutes intervals and maintained at the maximum tolerated dose for 5 consecutive hours for two consecutive days. The intervals between the infusions were between 6-8 weeks in both protocols depending on clinical response and availability of places at the Unit. 44 patients who received long term IV ILO as inpatients (Cohort A), after a wash out of 3 months, were switched to ambulatory administration (Cohort B). Thereafter, after one year of follow-up 24 patients of the protocol B were lost to follow-up and 20 patients were maintained on this regimen. Comparison was made between Cohort A (44 patients=A44) in the period between march 2015 and October 2016 (period A), Cohort B (44 patients=B44) between October 2016 and March 2017 (period B44), Cohort B (20 patients=B20), between October 2017 and March 2018 (period B20). Comparison between groups was made by non parametric tests and contingency table analysis when appropriate. Costs were estimated multiplying data related to resource use (drug consumpion, hospital stay, outpatient visits, management of complications, etc) by unit cost obtained from the accounting office of the Hospital. All costs were expressed in Euro Results Mean number of DUs at the end of period A was 0,37±0,98 in A44 as compared to 0,90±1,39 of period B44 cohort B44 (p=0,045) and 0,55±0,95 of period B20 Cohort B20 (p=n.s.). Cumulative number of DUs was 9/44 in Cohort A (20.5%) as compared to 20/44 in Cohort B44 (45% p=0.002) and 7/20 in cohort B20 (35% p=0,05). High number of drop outs (24/44) in protocol B was due mainly to statistical significant difference in tolerability (adverse events 11% protocol A vs 42% protocol B p Conclusion Protocol A seems to represent a regimen better tolerated and more effective than protocol B. Moreover, protocol B is affected by high rate of drops out due mainly to worse tolerability. Admitting SSc-patients for continuous IV ILO infusion seems to represent a better tolerated and more effective choice than following them as outpatients at infusion clinic, with substantially comparable direct costs in the same SSc cohort. Disclosure of Interests Alessandra Della Rossa: None declared, Simone Barsotti: None declared, Marco Di Battista: None declared, Lorenzo Puccetti: None declared, Valentina Lorenzoni: None declared, Giuseppe Turchetti: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB

Background: Ultrasonography of major salivary glands (SGUS) has been proposed as a feasible and reliable tool for the assessment of parotid and submandibular glands inflammation and damage in Sjogren’s syndrome (pSS). However, the relationship between major SGUS and labial salivary gland (LSG) histopathology is still unclear. Objectives: to investigate the correlation between SGUS abnormalities and LSG histopathology in patients newly diagnosed patients with pSS Methods: Consecutive patients suspected of having pSS were enrolled in this study. All the patients underwent a complete diagnostic work up. The echostructure of each gland on B-mode images was graded on a 5-point scale (0–4), and a SGUS score ≥2 was defined as pathological. Hypo-anechoic areas in the glands were defined as isolated ( 50%). Hyperechoic bands present in more than 50% of the parenchyma were also recorded. An expert pathologist analyzed all the LGS samples assessing focus score (FS), number of foci and presence of ectopic germinal centers (GCs). Results: We included 115 pts (105 F: 10 M, mean age (SD) 54.8 (12.5) years). Out of them 51 were diagnosed with pSS and 64 represented the no-SS sicca controls. A SGUS score ≥2 identified pSS patients with a sensitivity of 62.7% and a specificity of 96.7%. SGUS score significantly correlated with FS (r=0.457**, p=0.000), number of foci (r=0.359**, p=0.000) and number of ectopic GCs (r=0.505**, p=0.000). However, the overall concordance between SGUS and LSG histopathology was moderate (Cohen’s kappa =0.574). Thus, we specifically focused on 19/51 pSS “discordant” cases who presented normal SGUS findings but focal lymphocytic sialadenitis in their LSG biopsy. These “discordant” patients tended to present a lower FS (1.7±0.5 vs 2.6±1.5, p=0.04) and a significantly lower number of foci in their biopsies (2.7±1.6 vs 4.6 ±2.9, p=0.01) when compared to patients with both abnormal SGUS findings and LGS focal lymphocytic sialadenitis. Conclusion: SGUS and LSG histopathology showed only a moderate correlation in patients with pSS. SGUS may be useful to identify pSS patients with a more severe inflammatory LSG infiltrates, ultimately contributing to promote non-invasive phenotyping of multiple subsets in pSS. Disclosure of Interests: Chiara Baldini: None declared, Francesco Ferro: None declared, Nicoletta Luciano: None declared, Gianmaria Governato: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB, Stefano Bombardieri: None declared, Valentina Donati: None declared

Background Recommendations for the treatment of systemic lupus erythematosus (SLE) were published by EULAR in 2008. Advances in treatment strategies and goals called for an update of these recommendations, capitalizing on strengths and experience from previous projects. Objectives To update the EULAR recommendations for the management of SLE. Methods Systematic literature review (01/2007-12/2017) followed by Delphi method to form questions, elicit expert opinions and reach consensus. Results Treatment in SLE aims at remission or low disease activity and prevention of flares. Hydroxychloroquine is recommended in all lupus patients, at a dose not exceeding 5mg/kg real body weight. During chronic maintenance treatment, glucocorticoids should be minimized to less than 7.5 mg/day (prednisone equivalent) and, when possible, withdrawn. Prompt initiation of immunomodulatory agents (methotrexate, azathioprine, mycophenolate) can expedite tapering/discontinuation of glucocorticoids. In persistently active or flaring extrarenal disease, add-on belimumab should be considered; rituximab may be considered in organ-threatening, refractory disease. Specific recommendations are also provided for the management of cutaneous, neuropsychiatric, haematological and renal disease (including the use of calcineurin inhibitors). SLE patients should be assessed for their antiphospholipid antibody status, infectious and cardiovascular diseases risk profile, and preventative strategies be tailored accordingly. Conclusion Updated EULAR recommendations provide physicians and patients with updated consensus guidance on the management of SLE, combining evidence-base and expert-opinion. Acknowledgement European League against Rheumatism Disclosure of Interests Antonis Fanouriakis Paid instructor for: Amgen, GSK, Speakers bureau: Abbvie, Enorasis, Genesis Pharma, Myrto Kostopoulou: None declared, Alessia Alunno: None declared, Martin Aringer Grant/research support from: Roche, Consultant for: AstraZeneca and Eli Lilly, Ingeborg Bajema Consultant for: GSK, John N. Boletis: None declared, Ricard Cervera: None declared, Andrea Doria: None declared, Caroline Gordon Grant/research support from: Sandwell and West Birmingham Hospitals NHS Trust have received funding from UCB to support research work done by my research group that was unrelated to any pharmaceutical product or clinical trial., Consultant for: I have provided consultancy advice and taken part in scientific advisory boards on the design and analysis of clinical trials and the management of lupus for GSK, EMD Serono and UCB. I have taken part in adjudication and safety monitoring committees for BMS., Speakers bureau: I have been paid by UCB for speaking at meetings., Marcello Govoni Paid instructor for: Pfizer, Roche, Speakers bureau: Pfizer, Abbvie, MSD, Roche, Eli-Lilly, Celgene, Sanofi, Janssen, Frederic Houssiau: None declared, David Jayne Grant/research support from: David Jayne has received research grants from Chemocentryx, GSK, Roche/Genentech and Sanofi-Genzyme. He has received consultancy fees from Astra-Zeneca, Boehringer-Ingelheim, Chemocentryx, Chugai, GSK, Infla-RX, Insmed and Takeda, Marios Kouloumas: None declared, Annegret Kuhn Grant/research support from: Biogen, Galderma, GlaxoSmithKline, LeoPharma, Speakers bureau: La Roche Posay, Janni Lisander Larsen: None declared, Kirsten Lerstrom: None declared, Gabriela Moroni: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB, Matthias Schneider Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Speakers bureau: Chugai, Josef S. Smolen Grant/research support from: AbbVie, Eli Lilly, Janssen, MSD, Pfizer Inc, Roche, Consultant for: AbbVie, Amgen, AstraZeneca, Astro, Celgene, Celtrion, Eli Lilly, GlaxoSmithKline, ILTOO, Janssen, Medimmune, MSD, Novartis-Sandoz, Pfizer Inc, Roche, Samsung, Sanofi, UCB, Speakers bureau: AbbVie, Amgen, AstraZeneca, Astro, Celgene, Celtrion, Eli Lilly, GlaxoSmithKline, ILTOO, Janssen, Medimmune, MSD, Novartis-Sandoz, Pfizer Inc, Roche, Samsung, Sanofi, UCB, Elisabet Svenungsson: None declared, Vladimir Tesar: None declared, Angela Tincani Consultant for: UCB, Pfizer, Abbvie, BMS, Sanofi, Roche, GSK, AlphaSigma, Lillly, Jannsen, Cellgene, Novartis, Anne Troldborg: None declared, Ronald van Vollenhoven Grant/research support from: AbbVie, Amgen, Bristol-Myers Squibb, GlaxoSmithKline, Pfizer, Roche, and UCB, Consultant for: AbbVie, AstraZeneca, Biotest, Bristol-Myers Squibb, Celgene, Crescendo, GlaxoSmithKline, Janssen, Lilly, Merck, Novartis, Pfizer, Roche, UCB, and Vertex., Speakers bureau: AbbVie, AstraZeneca, Biotest, Bristol-Myers Squibb, Celgene, Crescendo, GlaxoSmithKline, Janssen, Lilly, Merck, Novartis, Pfizer, Roche, UCB, and Vertex., Jorg Wenzel Grant/research support from: GSK, Incyte, Consultant for: Biogen, Leo, Paid instructor for: Novartis, George Bertsias: None declared, Dimitrios Boumpas: None declared

Background: Renal involvement in systemic lupus erythematosus (SLE) is the most frequent severe manifestation and carries a bad prognosis. Objectives: To analyze the outcome of lupus nephritis (LN) in terms of chronic kidney disease development (CKD). Methods: Design: multicentre restrospective observational study. Patients: SLE patients (ACR97) with biopsy proven LN attending to three South European Rheumatology departments. Variables: demographics, SLE related variables, including global activity (SLEDAI-2K), renal flares, therapies, ACR response criteria and CKD. Statistical analysis: descriptive bivariate and multivariate analysis exploring factors associated to CKD. Results: Seventy-six patients with biopsy-proven LN were included, 90,7% female; mean age: 33 years; mean disease: duration 14 years; mean follow-up time (since LN diagnosis): 8,5 years. LN class III, IV and V were present in 22%, 75% and 3% of the cases, respectively. At LN diagnosis 68 (89%) patients had a severe renal flare. Forty-one (56.1%) and 49 (64.4%) had HTA and nephrotic syndrome, respectively. The mean 24h proteinuria levels at LN diagnosis was 4,6g. Mean SLEDAI-2K at the time of flare was 20.3, with 69 (65.7%) patients having an extrarenal flare. The treatments used to induce remission were: glucocorticoids (100%): pulses M-prednisolone in 49 (64%) and oral prednisone (mean starting dose): 43 mg/day (±20.6); intravenous cyclophosphamide in 42 (55%) patients; mycophenolate mofetil (MMF) in 21 (27%) patients; calcineurin inhibitors in 5 (11%) patients; rituximab in 4 (5.2%) patients; and oral cyclophosphamide in 4 (5,2%) patients. Forty-eight (63%) patients were receiving hydroxychloroquine. MMF was the immunosuppressant (IS) more frequently used (52%) as maintenance therapy. At 3, 6 and 12th months, the mean proteinuria was 2.3g/24h, 1.53g/24h, 1.1g/24h, respectively (p In the bivariate study, the following variables were significantly associated with CKD: male sex, hypertension, ACEI drugs, severe infection after LN, temporal dyalisis, non ACR renal response, non use of hydroxychloroquine, time to achieve 10mg/day of prednisone, previous creatinine to LN, maximum creatinine at LN, hyperlipidemia at 3 months of LN, active urinary sediment at 12 months, creatinine at 6 and 12 months, proteinuria at 6 and 12 months. In the logistic regression model, using genetic algorithms, we found that proteinuria at 6 months was significantly associated with CKD (OR:2.95; 95%CI 1.19,9.29, p= 0.03). Hypertension and male sex were marginally associated (p=0.06, both). Conclusion: A considerable percentage of LN patients developed renal chronic failure (21,9%). A high percentage of ACR response was achieved using medium dose (40mg) of glucocorticoids for induction. A significative reduction of proteinuria was achieved at 3 months, but proteinuria at 6 months was the only factor finally associated with CKD. Disclosure of Interests: Irene Altabas Gonzalez: None declared, Jose M Pego-Reigosa: None declared, Jose Maria Gonzalez: None declared, Francisco Rubino: None declared, Chiara Stagnaro: None declared, Chiara Tani: None declared, Carlos Rodriguez-Lozano: None declared, Maria Celia Erausquin: None declared, Juan Carlos Quevedo-Abeledo: None declared, Inigo Hernandez: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB, Inigo Rua-Figueroa: None declared

Background: Since many years, muscular magnetic resonance imaging (MRI) has been used in the diagnosis and follow-up of patients with idiopathic inflammatory myopathies (IIM), but the clinical significance and the pattern of the muscular alterations (edema, fatty infiltration and atrophy) in different subsets of the disease are still not well defined. Objectives: The aims of this study were to analyse possible differences in the involved muscles between dermatomyositis (DM) and polymyositis (PM); the correlations between the pattern of muscular involvement in different subsets of the disease and disease parameters in a monocentric cohort of patients. Methods: We retrospectively collected data from 85 patients with poly and dermatomyositis (EULAR/ACR criteria) who performed pelvic and tights muscle MRI from January 2010 to December 2018: 27 had DM and 58 PM, mean age 58.6±13.4 years, mean disease duration of 45±73 months. The images in 22 muscles for the presence of muscular edema, fatty infiltrates and atrophy were assessed by a dedicated radiologist. Moreover, data about serum creatine kinase (CK) and manual muscle test 8 (MMT8) were collected. Data were reported as median±interquartile range; non-parametric tests were used for the analysis. Results: Sixty-three patients presented muscular edema in at least one muscle. Patients with muscular edema had higher CK levels (1506±1976 vs 235±224 p Forty-eight patients presented fatty infiltrates that were more frequent in older patients (63.1±11-3 vs 52.8±13.7 p=0.01) and in those with longer disease duration (39.1±95.4 vs 9.5±12.5 p=0.024). CK levels and MMT8 were not different in patients with or without muscular fatty infiltrates. With the multivariate analysis the disease duration represents the only independent factor for the presence of muscular fatty infiltrates (p=0.05). Muscular atrophy was present in 17 patients but it was not correlated to age and disease duration. CK and MMT8 were not different in presence/absence of fatty infiltrates or muscular atrophy. Edema and atrophy were not different between poly- and dermatomyositis. The fatty infiltrate was more present in the posterior compartment of the tights (biceps femoris and semitendinosus muscle) in patients with PM compared to DM (68.2% vs 29.6% p=0.046). Conclusion: The alterations identified with MRI in our cohort changed according to the disease duration and the age of the patients. In particular, fatty infiltration was more frequent in patients with longer disease duration, but was not associated to CK levels and muscular weakness. Moreover, fatty infiltration was prevalent in the posterior compartment of the tights in PM patients. Muscular MRI is widely used in the diagnosis and follow-up but the clinical meanings of the different alterations still need to be investigated. Disclosure of Interests: Simone Barsotti: None declared, Barbara Mugellini: None declared, Alessandra Tripoli: None declared, Giacomo Aringhieri: None declared, Chiara Cardelli: None declared, Elisa Cioffi: None declared, Virna Zampa: None declared, Davide Caramella: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB, Rossella Neri: 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 Interstitial Lung Disease (ILD) is one of the most frequent and significant extra-muscular manifestations in Idiopathic Inflammatory Myopathies (IIM). Although high resolution computed tomography (HRCT) remains the gold standard technique for the evaluation of ILD, during the last years several authors proposed ultrasound (US) quantification of pleural irregularities (PIs) as a possible method to detect lung involvement in IIM patients. Objectives The objective of our study was to evaluate the prevalence of PIs and to compare the results obtained to the data provided by HRCT. Methods Thirtyseven patients (24 females,13 males, mean duration of the disease 5.21±7.6 and median age 62.24±1.8 years) with a diagnosis of IIM according to Bohan and Peter criteria (17PM,16DM,1MCI,3overlap syndromes) who required HRCT evaluation were enrolled. All patients underwent rheumatological clinical evaluation, including dyspnoea measurement by means MRC scale and disease activity assessment according to IMACS criteria (muscle enzymes, MMT8, VAS, HAQ, MYOACT). Patients were also asked to complete Patient Reported Outcome (PRO) questionnaires as Leicester Cough Questionnaire (LCQ). All the patients underwent a thoracic US evaluation in 53 anterior and posterior thoracic areas by Esaote MyLab Gold ultrasound device with 8–18 MHz linear probe. We evaluated the aspect of the pleural profile assigning each space a score according to a 3 points scale (regular=0, mild irregularity=1, irregularity=2) and summed the score in each space to obtain the PIs total score. HRCT was assessed by an expert radiologist to obtain a semiquantitative evaluation of parenchymal involvement by Warrick score. In a subgroup of patients, thoracic US was repeated by the same operator after two days from the first evaluation and by a second operator to valuate the intrareader and interreader agreement of the technique. Results The PIs total score obtained with lung US (24.70±13.66) was higher in patients with thoracic crackles at clinical examination (p=0.008) and in patients with positivity for antisynthetase autoantibodies, particularly anti-Jo1 (p Conclusions The US PIs total score is strictly correlated to clinical, serological and HRCT parameters and as the lung US is a non-invasive and relatively inexpensive technique, the results of our study suggest a possible role of this method in the screening of lung involvement in IIM patients. Follow up studies in a larger cohort of IIM patients are required. Disclosure of Interest None declared

Background Musculoskeletal symptoms are frequently reported by patients with inflammatory bowel diseases (IBD). Those symptoms may vary from arthralgia to arthritis, involving peripheral or axial joints, with a prevalence ranging from 17% to 62% when considering any SpA manifestation (with a similar range for axial or peripheral involvement from 5% to 30%) and a definite SpA diagnosis up to 46%.1–10 To date, a more comprehensive approach is needed, also because the use of DMARDs or biologics could improve both gastrointestinal and musculoskeletal symptoms. Objectives To evaluate the rate of MSK involvement in a mono centric cohort of IBD patients. Methods A questionnaire based on the features of SpA was used in the IBD out-patient clinic from January 1st to December 31st 2017. When there was a positivity for any feature, the patients were evaluated by a rheumatologist (with more than 18 years of experience in SpA). Al the visits were performed in 2–3 weeks from the moment the questionnaire was performed (varying according to the seasonal time for holidays). When there were some doubts about the diagnosis, further examinations were requested. Results A total of 403 patients were visited in the out-patient clinic (220 affected by CD, 172 affected by UC and 11 with a not defined IBD). Fifty-nine patients were sent to the rheumatologist (33 CD, 24 UC and 2 not defined IBD). Eleven patients had 2 or more rheumatologic visits (to follow-up the disease and check the results of the exams requested). To allow a diagnosis, 4 sacroiliac joints MRI and 1 ultrasound assessment of the feet were requested. A diagnosis of peripheral SpA was given in 9 patients while axial SpA was diagnosed in 7 subjects. The diagnosis was fibromyalgia, osteoarthritis and arthralgia in 1, 2 and 40 patients, respectively. Therapy was modified in 16/59 patients after the rheumatologic assessment (DMARDs were prescribed in 12 subjects while anti-TNFa in 4 of them). In 1 patient (with absolute contraindication for biologic therapy), two courses of SI joint injection were performed, improving local pain. Conclusions The results of our study confirm the already published prevalence of musculoskeletal involvement in IBD patients (15% of our IBD population complained musculoskeletal pain and 27% of the patients sent to the rheumatologist were given an enteropathic arthritis diagnosis). As for the already existing literature, we did not notice any evident difference in the prevalence of axial and peripheral involvement. An established collaboration between gastroenterologists and rheumatologists is necessary to provide an integrated and more comprehensive management of IBD, improving the quality of life of the patients.11–12 References [1] Palm O, et al. J Rheumatol2002. [2] Salvarani C, et al. Scand J Gastroenterol2001. [3] Olivieri I, et al. Autoimmun Rev2014. Disclosure of Interest None declared

Background Anti-MDA5 antibodies are a known set of antibodies observed mainly in dermatomyositis, typically associated with cutaneous involvement, presence and often rapid progression (RP) of interstitial lung disease (ILD) and amyopathic muscle involvement. Despite the increased attention, described cohorts involves only a limited number of cases Objectives to define clinical characteristics of a large cohort of anti-MDA5 antibodies positive patients Methods Retrospective assessment of anti-MDA5 positive patients from centres referring to our group Results 82 anti-MDA5 positive cases (56 females) were collected. In median: onset age was 44 Y (IQR 22.57), diagnostic delay 4 Mo (IQR 1–10), follow-up 13 Mo (IQR 3–43). Fifty-three patients had ILD, that was RP in 24 cases (15 at ILD onset, 9 after ILD onset). Fifthteen ILD patients were admitted in Intensive Care Unit (ICU): 4 were treated with Extracorporeal-Membrane-Oxygenation, 7 with mechanical-invasive and 2 with mechanical-non-invasive ventilation. Forthy-nine patients had muscle involvement (39 symptomatic), 42 arthritis, 54 cutaneous involvement, 9 history of malignancy. Twelve patients died (ILD=7, ILD and sepsis=3, neoplasia=1, not specified=1), 7 of those in ICU Conclusions In our cohort, ILD was the most frequent finding. A RP of ILD was common, occurring also in ILD with symptomatic/chronic onset. The low rate of survival in ICU raised the problem of follow up and early treatment of ILD. In our cohort arthritis was common and muscle involvement mainly symptomatic. Finally, the high percentage of observed malignancies suggests a careful neoplastic screening and follow up Reference [1] Labrador-Horrillo M, et al. J Immunol Res2014;2014:290797. Disclosure of Interest None declared

Background Behcet’s syndrome (BS) is a systemic vasculitis, typically characterised by recurrent oro-genital ulcers, ocular inflammation and skin manifestations; articular, vascular, gastro-enteric and neurological involvement may also occur.The complex pattern of BS profile can effect negatively on patients‘ quality of life. Objectives The primary aim of this study was to explore the role of quality of life in BD patients by means of patient reported outcome (PRO); the secondary aim was to study any correlation between disease activity and quality of life. Methods The study enrolled 130 patients (71 M, 59 F), all fulfilling the International Study Group (ISG) criteria for BS. Their mean age was 42±8 years, 18–77 while the mean age at disease onset was 11±4 years 5–18 and the mean follow-up of 8±2 years. Disease activity was evaluated by means of the Behcet’s Disease Current Activity Form (BDCAF), while Short-form-36 (SF-36) was used to evaluate quality of life. Disease activity was compared with the global SF-36 score and with each dimension, that includes: physical functioning, physical disability, body pain, general health, vitality, social functioning, emotional disability, mental health. The statistical analysis was performed using Student t-test, Mann-Whitney-U test, ANOVA and Pearson correlation Results At time of evaluation, according BDCAF, 51 BS patients (39%) had clinically active disease (36 muco-cutaneous involvement, 21 ocular involvement 10 joint involvement, 6 neurological involvement, 4 gastro-enteric). As expected, the overall SF-36 scores were significantly lower in patients with clinically active disease. Moreover, female BS patients had statistically significant lower scores in all SF-36 domains compared with male patients. When each domain of SF-36 was evaluated, we found that physical disability (p=0.004), body pain (p=0.006), general health (p=0.001), and vitality (p=0.001) were significantly lower in patients with disease activity. Notably, vitality (p=0.001), physical disability(p=0.004), social functioning (p=0.001), emotional disability (p=0.003) and mental health (p=0.001) were significantly lower in patients with muco-cutaneous active disease, compared with the other patients with active disease. Conclusions The clinicians who take care of any chronic disease would like to correctly know the current status of a patient to manage him properly. In this regard, the combination data of PRO measures and disease activity have been demonstrated to add more information compared to the evaluation of disease activity alone. These consideration suggest that the correct assessment of BS needs a multi-dimensional approach, that fairly includes disease activity, disease damage and quality of life Disclosure of Interest None declared

Background Preventing organ damage is a major challenge in Systemic Lupus Erythematosus (SLE). Objectives To evaluate factors associated with development of damage in a prospectively followed cohort of early diagnosed SLE patients. Methods The Early Lupus Project1 encompasses 9 Italian centres recruiting, from the 1 st January 2012, an inception cohort of consecutive patients diagnosed with SLE within 12 months from appearance of four or more 1997 ACR classification criteria. At study entry and then every 6 months a large panel of data was recorded. Here, we report on factors associated with the development of damage assessed by the SLICC/ACR Damage Index (SDI). Using univariate analysis, we assessed the contribution of covariates collected at baseline (demographic, comorbidities, serological, clinical by BILAG2004 domains, disease activity by ECLAM, HRQoL by visual analogic scale) in the development of damage (SDI from 0 to ≥1). Forward-Backward Cox-regression models were fitted with covariates with p Results Overall, 279 patients were enrolled in the Early Lupus Project inception cohort up to the 31th of December 2017; 230 patients (89.6% Caucasians, 13.4% males) were eligible for this study having SDI=0 at enrolment and at least 6 months of follow-up. Mean (±SD) age at recognition of 4 ACR criteria was 36.5 (±14.4) years, median disease duration at recruitment was 1.1 months (interquartile range 0.0–4.8) and median follow-up duration was 27.4 months (interquartile range 7.2–48.0). At last follow-up visit 84 patients (36.5%) had an SDI score ≥1 (median=0; interquartile range 0–1); see figure 1A for overall SDI domains involved. Baseline dyslipidemia (p 35 year) at baseline (p=0.001; HR 2.3 95% CI 1.4–4.0) together with total dose of corticosteroids (p=0.015; HR 1.06 per gram of prednisone equivalent; 95% CI 1.01–1.11) during follow-up were the factors independently associated with increased risk of developing damage in this cohort (figure 1B). Their effect was confirmed after stratification for antimalarials (yes/no) and immunosuppressants (yes/no) use. Conclusions The early development of organ damage in this SLE patients cohort was associated with modifiable risk factors as baseline dyslipidemia and higher corticosteroid dose. Addressing them since the very early stages of the disease, and treating disease activity to target remission or minimal disease activity, may reduce damage and improve patients outcome. Reference [1] Sebastiani GD, et al. Early Lupus Project – A multicentre Italian study on systemic lupus erythematosus of recent onset. Lupus2015Oct;24(12):1276–82. Disclosure of Interest None declared

Background Baricitinib (Bari), an oral selective inhibitor of Janus kinase (JAK)1 and JAK2, has been approved for the treatment of RA in the EU and Japan. Objectives To report results from a 24 week (wk) global, Phase 2, double-blind, placebo (PBO)-controlled study of Bari in patients with SLE receiving standard therapy. Methods Patients with SLE (positive ANA or anti-dsDNA, clinical SLEDAI-2K≥4, arthritis or rash required) receiving stable background SLE therapy were randomised 1:1:1 to PBO, or Bari (2- or 4 mg) once daily. The primary endpoint was resolution of SLEDAI-2K arthritis or rash at wk 24. Results Of 314 patients randomised, 79%, 82%, and 83% completed 24 wks of treatment in PBO, Bari 2 mg, and Bari 4 mg groups, respectively. At wk 24, a significantly greater proportion of patients in Bari 4 mg group compared to PBO achieved resolution of SLEDAI-2K arthritis or rash (67% vs 53%, p Data are n (%) patients, unless otherwise indicated. D=least squares mean change from baseline. ‡Includes up to 30 days post treatment. CLASI=Cutaneous Lupus Erythematosus Disease Area and Severity Index; n=number of patients in the analysis population; n=number of patients in the specified category; TEAE=treatment emergent adverse event.*p≤0.05. Conclusions In patients with SLE receiving standard background therapy, once-daily oral Bari 4 mg was associated with significant clinical improvements compared to PBO and an acceptable benefit/risk profile. These findings support further study of Bari 4 mg as a potential therapy for patients with SLE. Disclosure of Interest D. Wallace Consultant for: Eli Lilly and Company, EMD Merck Serono, Pfizer, GSK, R. Furie Consultant for: Eli Lilly and Company, Y. Tanaka Grant/research support from: Mitsubishi-Tanabe, Takeda, Bristol-Myers, Chugai, Astellas, Abbvie, MSD, Daiichi-Sankyo, Pfizer, Kyowa- Kirin, Eisai, Ono, Speakers bureau: Daiichi-Sankyo, Astellas, Pfizer, Mitsubishi-Tanabe, Bristol-Myers, Chugai, YL Biologics, Eli Lilly, Sanofi, Janssen, UCB, K. Kalunian Consultant for: Eli Lilly and Company, M. Mosca: None declared, M. Petri Consultant for: Eli Lilly and Company, T. Dorner Grant/research support from: Roche/Chugai, Janssen, Sanofi, Consultant for: AbbVie, Celgene, Eli Lilly, Roche, UCB, MSD, Pfizer/Hospira, Novartis, Speakers bureau: Amgen, Celgene, Biogen, M. Cardiel Grant/research support from: Pfizer, Gilead, Roche, Janssen, Consultant for: Eli Lilly and Company, Pfizer, Speakers bureau: Eli Lilly and Company, Pfizer, Abbvie, I. Bruce Grant/research support from: Genzyme, GSK, Consultant for: BMS, Eli Lilly and Company, GSK, Astra Zeneca, Speakers bureau: GSK, E. Gomez Shareholder of: Eli Lilly and Company, Employee of: Eli Lilly and Company, T. Carmack Shareholder of: Eli Lilly and Company, Employee of: Eli Lilly and Company, J. Janes Shareholder of: Eli Lilly and Company, Employee of: Eli Lilly and Company, M. Linnik Shareholder of: Eli Lilly and Company, Employee of: Eli Lilly and Company, M. Silk Shareholder of: Eli Lilly and Company, Employee of: Eli Lilly and Company, R. Hoffman Shareholder of: Eli Lilly and Company, Employee of: Eli Lilly and Company

Background Interstitial lung disease (ILD) is a possible manifestation of several rheumatic diseases. Although lung involvement is common in Anti-neutrophil cytoplasmic antibodies (ANCA) vasculitides (AAV), the presence of ILD is relatively rare. Moreover, a very small subgroup of patients may present ILD along ANCA positivity without other systemic signs of AAV. Objectives To describe the phenotypic characteristics of ILD patients with ANCA positivity without a definite diagnosis of AAV. Methods ANCA-ILD patients from 7 tertiary care hospitals (6 from Italy, 1 from Spain – 2 rheumatology centres, 4 pneumology centres, 1 internal medicine) were included. The mean follow-up was of 62.6±49.8 months. We collected epidemiologic, demographic, clinical, serological, pulmonary function test (PFT) data and high resolution computed tomography (HRCT) pattern. Results 19 patients with ANCA-ILD (M:F=9:10, mean age at diagnosis 66.2±8 years) were enrolled. The mean latency between the first respiratory symptom onset and the diagnosis was 26.4±37.0 months. Sixteen patients (84%) had ANCA-MPO positivity while 3 ANCA-PR3. Antinuclear autoantibodies were positive in 12 patients (63%). Usual interstitial pneumonia was the main HRCT pattern (10, 52.6%), followed by non specific interstitial pneumonia (8, 42%, 1 associated with organising pneumonia), 1 combined pulmonary fibrosis and emphysema. Six patients with ILD-ANCA presented also a non-erosive oligoarticular inflammation (n=6). During the follow-up, the majority of the patients (16/19) did not require oxygen therapy at last evaluation. However, 1 patient required continuous O2, 1 intermittent O2, 1 patient underwent lung transplant due to respiratory failure. Five patients required hospitalisation due to respiratory insufficiency. Two patients died, both for respiratory insufficiency. All patients were treated with glucocorticoids as induction therapy (4 intravenous pulses), combined with an immunosuppressant in 11 cases: cyclophosphamide (3, 15%), azathioprine (6, 31%), mycophenolate (2, 10.5%), methotrexate (1, 5.5%) or rituximab (2, 10.5%). Conclusions the observation of ANCA positivity in patients with ILD may open new prospective in the assessment of interstitial lung diseases. Although most of patients had a good prognosis, up to 25% presented unfavourable respiratory outcome. Further prospective studies in larger cohorts and longer follow-up may clarify the prognosis of this particular lung disease and if ILD-ANCA should be classified as a distinct subset or an incomplete form of AAV. Disclosure of Interest None declared

Background This 4 phase project1 jointly supported by EULAR and ACR has led to draft criteria.2 Objectives To simplify and validate the new criteria in a large international cohort. Methods 23 expert centres each contributed up to 100 patients with SLE and with non-SLE diagnoses. Diagnoses were verified by 3 independent reviewers for 1,193 SLE and 1059 non-SLE patients. 500 randomly selected SLE and non-SLE patients formed the derivation cohort and the remainder the validation cohort. Results The criteria were fine-tuned and simplified, using ANA of ≥1:80 as entry criterion and a classification threshold of 10. Sensitivity was close to the SLICC 2012 criteria, specificity maintained at the level of the ACR 1997 criteria. This performance was independently confirmed in the validation cohort. Conclusions The new criteria developed with EULAR/ACR support achieved sensitivity close to the SLICC criteria, while maintaining the specificity of the ACR criteria. References [1] Aringer, et al. Ann Rheum Dis2017;76(S2):4. [2] Tedeschi, et al. Ann Rheum Dis2017;76(S2):50. Disclosure of Interest None declared

Background Cardiovascular (CV) disease is a major cause of mortality and morbidity in SLE patients; several instruments have been developed to estimate the 10 years risk of major CV events in the general population according to the presence of traditional CV risk factors. However, it is well known that traditional CV risk factors cannot fully explain the increased CV disease in SLE. Recently, a new version of the Qrisk algorithm was released (QRISK3) incorporating new variables associated with increased risk of CV disease, including SLE diagnosis and the GC use. Objectives To assess the 10 years CV risk in a cohort of SLE patients by comparing three different algorithms. Methods Consecutive patients with a diagnosis of SLE according to the ACR criteria were recruited from the inpatients and outpatients clinics of our Unit. Previous major CV events (myocardial infarction and stroke) were considered exclusion criteria. Traditional CV risk factors, disease activity and damage as well as therapies and comorbidities were evaluated. Disease activity was assessed with the SLEDAI score, organ damage with the SLICC/DI. Three algorithms to estimate the 10 years risk of major events were applied: the Framingham score, the ACC/AHA score and the QRISK3. This latter incorporates new variables associated with increased risk of cardiovascular disease, including SLE and GC use. Results 115 SLE patients (90.5%) without previous major CV events were included in this analysis. They were predominantly females (93%), Caucasian (96.5%) with a mean age at enrollment of 42.8±12.5 and a mean disease duration of 13.4±9.2 years. The prevalence of organ involvement and traditional risk factors are reported in table 1. Disease activity at enrollment was low (median SLEDAI 2, IQR 0–4) while 39.1% was presenting at least one organ damage. The median Framingham risk score resulted 4.5% (IQR 2.4–8.6), the ACC/AHA resulted 1.2% (0.5–3.2), the QRISK3 resulted 5.4% (2.4–9.6). According to the three methods, there was a significant difference in the risk stratification: 10% of patients at medium-high CV risk according to the Framingham score, 5% with the ACC/AHA score and 21% with the QRISK3. The median “healthy heart age” of our cohort (the age at which a healthy subject has the same CV risk) provided by the QRISK3 resulted 62 years.52–68 Conclusions These data show that by applying the QRISK3 algorithm a significant percentage of patients would be reclassified at high risk of CV events with respect to the traditional CV risk scores. Longitudinal data are necessary to validate the QRISK3 accuracy against the traditional scores in the identification of SLE patients at higher risk of CV events. Disclosure of Interest None declared

Background High resolution computed tomography (HRCT) is an essential technique for the characterisation of interstitial lung disease (ILD) in patients with idiopathic inflammatory myopathies (IIM). Although several visual scores are available for a semiquantitative evaluation of ILD, an automatic quantitative analysis of lung involvement may represent a valuable improvement to reproducibly determine the extent of the disease. Objectives To compare the semiquantitative visual score to a volumetric texture and local volumetric histogram feature-based analysis software (CALLIPER) to quantify the lung involvement in IIM patients. Methods 21 consecutive IIM patients (Bohan and Peter criteria) who underwent a HRCT between November 2016 and November 2017 were prospectively enrolled. Twelve were affected by polymyositis and 9 by dermatomyositis. We collected smoking habits data and myositis specific autoantibodies positivity, respiratory symptoms according to MRC dyspnea scale and two patients reported outcome questionnaires: Leicester cough questionnaire (LCQ) and St. George’s Respiratory questionnaire (SGRQ). Patients underwent a non-contrast, supine, volumetric CT (≤2 mm slice thickness) that was evaluated by an expert radiologist according to Warrick’s score (WS) and also quantified by CALLIPER (Mayo Biomedical Imaging Resource – USA). CALLIPER analysis of the extent of interstitial lung abnormalities (ground glass, lung reticulation, honeycombing) was performed and a total score, expressed as percentage of lung involved (ILD%), was obtained. Analysis of the vascular involvement were performed as percent of pulmonary vessel volume (VESS%). Results Between the 21 patients, five (23.8%) were smokers. Four patients were positive for anti-synthetase autoantibodies -ASS (3 Jo-1, 1 Pl-7), 1 SRP, 1 Mi2. Dyspnea was present in 19 patients (90%): 9 grade 1, 7 grade 2, 3 grade 3 according to MRC scale. There were strict correlations between WS and ILD (r=0.802 p Conclusions CALLIPER represents an innovative technique to quantify the lung involvement in IIM patients: the main advantage of CALLIPER is the reproducibility that avoids the inter and intra-reader bias. Although more data are needed in larger cohorts, the use of CALLIPER may open new routes for the evaluation of ILD in IIM patients, both in medical practice and in randomised controlled trials. 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

Background Behcet’s syndrome (BS) is a multisystemic, chronic relapsing inflammatory disease classified among the vasculitis. Eye involvement represent one of the most serious manifestation of BS and occurs in half of all patients. It seems more frequent and severe among young males and, unluckily, it still represents a significant cause of morbidity. Objectives The aims of the study were: to study the relapse rate of BS patients with ocular involvement and to identify factors able to predict long-term outcome Methods Among a cohort of 138 patients with a diagnosis of BS according the ISG criteria, 62 patients (37 males and 25 females; mean age at the onset 38±5 years) with ocular involvement were prospectively studied. The probability of clinical relapse after remission of the first ocular attack was calculated using the Kaplan-Meier method. Predictors of long-term outcome were identified by univariate analysis using the log-rank test and by multivariate analysis using Cox proportional hazards regression models. Results The mean time between the first initial symptoms of BS and the onset of eye lesions was 2±2 years. The number of ocular attacks were the following: 43 posterior uveitis, 27 anterior uveitis, 26 retinal vasculitis, while panuveitis developed in 18 subjects. The cumulative relapse rates at 1 year, 3 years, and 5 years after remission of the first ocular attack were 41%, 31%, and 28%, respectively. On multivariate analysis, a younger age ( Conclusions The relapse rate seems to be more frequent in the first years of diseases, and probably it could be related to the fact the disease tend to grow dim over time. As literature data suggest, younger age and male sex still represent predictive factors of poor long-term clinical outcome. Disclosure of Interest None declared

Background Salivary gland ultrasonography (SGUS) has increasingly appeared as a valid tool to characterise major salivary glands involvement in primary Sjogren’s syndrome (pSS). An international score based on the number and location of the hypoechoic areas in the gland has been proposed for diagnostic purposes. Recently, a specific interest in using SGUS to estimate major salivary gland damage has also arisen in order to individualise patients treatment. Objectives a) to investigate frequency and severity of salivary gland damage in a large monocentric cohort of pSS patients; b) to explore determinants that could be associated with salivary gland damage accrual. Methods Sonographic data from a monocentric cohort of 311 pSS patients were collected from 2012 to 2017. SGUS had been performed according to a standard protocol that evaluated the echostructure of each gland graded on a 5-point scale (0–4). Salivary gland damage was defined by the presence of hyperechoic bands in more than 50% of the glandular parenchyma and on the basis of the size of the four glands. Results We included in the study 311 patients. The median age of the patients was 58 years (IQR, 49–68) and the median disease duration was 3 years (IQR, 0–9). Out of the cohort, 163 (52.4%) patients underwent SGUS examination within 3 years from the diagnosis, 91 (29.3%) within 4 to 10 years and 57 (18.3%) 10 years or more after the diagnosis. At SGUS evaluation 134/311 (43.1%) pSS patients did not present any findings of damage, whereas 177/311 (56.9%) presented at least one element of damage. SGUS damage inversely correlated with USFR (r=-0.382, p-value=0.001). At the univariate analysis SGUS damage was associated to disease duration, number and location of the hypo-anechoic areas, focus score, ESSDAI score, hypergammaglobulinemia, and positivity for anti-Ro/SSA, anti-La/SSB, Rheumatoid Factor (RF) and cryoglobulins. In the linear regression, duration of the follow-up, number and location of the hypo-anechoic areas and presence of cryoglobulins remained independently associated with SGUS damage. In 138/311 (44.4%) patients, SGUS was repeated prospectively at least twice during a median follow-up of 24 months (IQR, 12–36). Notably, 23/138 (16.7%) patients presented a damage progression. Presence of hyperechoich bands in the submandibular glands at the baseline evaluation was an independent risk factor for damage progression, whereas pSS low disease activity (ESSDAI Conclusions Ultrasonographic damage resulted quite frequent in pSS patients and significantly associated with salivary gland dysfunction. Notably, pSS patients with normal echostructure of their glands were those less prone to develop SGUS damage during the disease course, whereas those presenting localised, diffuse or scattered hypo-anechoic areas were at higher risk of damage accrual. Disclosure of Interest None declared

Background Primary Sjogren’s Syndrome (pSS) is a chronic progressive disease potentially leading to irreversible organ damage. To date only a limited number of studies have analysed prevalence and factors associated with damage accrual in pSS. Objectives a) to characterise cumulative damage in pSS patients, b) to identify determinants associated with its presence and c) to evaluate the impact of damage on patient reported outcomes (PROs). Methods Data from a monocentric cohort of 466 pSS patients were analysed. Glandular and extra-glandular damage manifestations were assessed by the Sjogren’s Syndrome Disease Damage Index (SSDDI). Additional items of damage defined ‘a priori’ as being potentially related to treatment (i.e osteoporosis, diabetes, infections) were analysed separately. The EULAR Sjogren’s Syndrome Disease Activity Index (ESSDAI) was used to measure disease activity at baseline and prospectively during the follow-up. The EULAR Sjogren’s Syndrome Patient Reported Index (ESSPRI), Oral Health Impact Profile (OHIP) and Ocular Surface Disease Index (OSDI) were utilised to record PROs. Patients’ comorbidities were assessed by the Charlson Comorbidity Index (CCI). Results A total of 466 pSS patients (446 F:20 M, median age (IQR): 59 years (48–69) were included in the study. The frequency of anti-Ro-SSA in the cohort was 69.5% (324/466). The median ESSDAI was 4 (IQR 1–8) at baseline and 2 (IQR 0–5) at the last evaluation, respectively. In addition to symptomatic agents, patients had been treated during the disease course with low-medium doses of glucocorticoids (GCs) (56%), hydroxychloroquine (HCQ) (62%) and DMARDs (16.6%). After a median follow-up of 5 years (IQR, 2–10), 208 patients (44.6%) had accrued some damage in either the oral damage items (33%), ocular damage items (20%) and/or systemic damage items (12%). In addition, 24/466 patients had developed a non-Hodgkin lymphoma and 2 patients a multiple myeloma. The SSDDI score ranged from 0 to 14. In the regression analysis: patients more likely to develop damage were those that were older, with a longer disease duration, higher baseline ESSDAI and who had been treated with DMARDs, whereas patients who had been ever treated with HCQ were less likely to develop disease-related damage. Similarly, treatment-related damage was independently associated with: disease duration, age of the patients, baseline ESSDAI, anti-Ro/SSA positivity and use of GCs. Oral and ocular damage items significantly correlated with ESSPRI, OHIP and OSDI, whereas systemic damage items positively correlated with patients CCI. Conclusions This large pSS cohort confirmed that demographic and clinical characteristics as well as medication are independently associated with disease-related and treatment-related damage. In particular, this study shows a highly significant impact of baseline disease activity on the development of future damage and poor PROs in pSS patients. Disclosure of Interest None declared

Background Evaluation of organ damage is one of the most important assessments in SLE patients; the SLICC/DI is a physician –driven instrument widely used to assess organ damage in SLE. Patient-reported outcomes (PROs), self-assessments that are not being interpreted by a clinician, are gaining a central role as outcome measures. Among these, the Brief Index of Lupus Damage (BILD) is a patient-reported instrument for the assessment of organ damage in SLE.1 Objectives The aims of the present study are translation, cultural adaptation and validation of an Italian version of the BILD. Methods The process of translation and cultural adaptation followed published guidelines.2 The final version of the questionnaire (BILDit) was pretested in a group of 30 SLE patients to evaluate acceptability, comprehension and feasibility. The validity of the BILDit was evaluated by its administering to consecutive patients attending the outpatients clinic or the inpatients wards. The external validity was tested toward the SLICC/DI scored by a physician blinded to the BILDit results. Correlation with other PROMs (FACIT, SF-36) was also tested. In a subgroup of 30 patients the questionnaire was administered twice at 2 weeks interval to assess their reliability. Results A total of 166 SLE patients participated to the study (93.4% female, 97% Caucasian); mean age (±standard deviation) at enrollment was 45.4 years±13, mean disease duration resulted 14.3±10 years. Overall, disease activity at enrollment as expressed by the SLEDAI score resulted quite low (median SLEDAI 2, IQR 0–4) and 27.3% of patients presented an active disease (SLEDAI >4). At least one organ damage according with the SLICC/DI was present in 111 patients (56.35%) with a median score of 2 (IQR1–4). In the pilot test, 80% of patients answered to all the questions; 93% of the patient declared no difficulty in completing the questionnaire and the median time required resulted 5 min.3–5 BILDit showed very high reliability (test-retest α >0.8). The BILDit scores in our sample showed a strong positive correlation with SLICC/DI (rho=0.69; p Conclusions BILDit demonstrated to be acceptable, comprehensible, feasible and reliable in our routine clinical setting; it also showed good correlation with physician’s driven instruments and quality of life measures. The BILDit can be considered a useful screening tool for the assessment of organ damage as perceived from the patient’s point of view before the standard visit. References [1] Yazdany, J. et al. The Brief Index of Lupus Damage (BILD): A patient-reported measure of damage in SLE. Arthritis Care Res. 2011; 63, 1170–1177. [2] Guillemin F. et al. Cross-cultural adaptation of health-related quality of life measures: Literature review and proposed guidelines. Journal of Clinical Epidemiology1993; 46: 1417–1432 Disclosure of Interest None declared

Background Hypereosinophilia-associated syndromes are a heterogeneous group of diseases characterized by sustained and elevated blood eosinophilia with evidence of eosinophil-induced organ damage. Classically, Eosinophilic granulomatosis with polyangiitis (EGPA) and Hypereosinophilic syndrome (HES) present several overlapping clinical and laboratory features, making it challenging to correctly insert patients in restricted and well-defined categories with specific and more effective therapeutic approaches. Therefore, great efforts are ongoing searching for novel biomarkers able to differentiate these two disorders in daily practice. Objectives To detect T cell receptor gamma (TCRG) clonal rearrangements in EGPA and HES, comparing the frequency distribution of V region and J region segment utilization in the study population. Methods In this single center study, we included consecutive patients with a diagnosis of EGPA and HES. Inclusion criteria were: documentation of a persistent peripheral eosinophilic count of ≥1.5 x109/L and signs or symptoms of organ involvement. Clinical and laboratory data of the patients were collected. Sequence-based determination of the frequency distribution of TCRG Gene Rearrangements was performed using next-generation sequencing with the Illumina MiSeq (LymphoTrack TRG assay, Invivoscribe). Results We included 21 patients (9 with EGPA and 12 with HES). Four EGPA patients were MPO-ANCA positive. We detected TCRG clonal rearrangements in 44% (4/9) patients with EGPA and in 42% (5/12) patients with HES (p-value = n.s). No association was observed between TCRG clonal rearrangements and ANCA status in EGPA patients. Recurrent TCRG gene rearrangements were observed; in particular, Vg10JgP1 (5 cases) and Vg4Jg1/2 (4 cases) were detected in both EGPA and HES, whereas Vg9Jg1/2 (2 cases) and Vg10Jg1/2 (2 cases) were found only in patients with HES. Conclusions Even if preliminary, this study reveals a similar T cell receptor gamma repertoire in EGPA and HES, thus suggesting a possible antigen-driven inflammatory response underlying hypereosinophilia in both EGPA and HES. Moreover, our results would suggest that the TCR clonality cannot be used as a tool for the differential diagnosis between EGPA and HES. Disclosure of Interest None declared

Background Celiac disease (CD) is an inflammatory and immune-mediated gluten-dependent enteropathy occurring in genetically susceptible individuals. CD is recognized to affect between 0.6% and 1% of worldwide population, with wide regional differences. Disease clinical features are protean and highlight the systemic nature of the disease. In recent years, an increased prevalence of CD has been also reported in patients with connective tissue diseases (CTDs). This association may be due to a shared genetic predisposition, to immunological mechanisms and/or exposure to a common triggering event. However, this observation remains controversial since data are usually based on descriptive case reports. Different methods of antibody detection and enrolled population sample size may contribute to result discordance. Moreover, CD diagnosis is often delayed because disease clinical spectrum may be atypical mimicking rheumatologic conditions and autoimmune disease itself may display typical symptoms of CD. Undoubtedly, awareness of CD occurrence in CTDs is important to prevent potential long-term systemic complications related to an unrecognized CD in these patients. Objectives To evaluate the prevalence of overt and subclinical CD and features of the disease in a large cohort of patients with systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and primary Sjogren9s syndrome (pSS) with a multicenter prospective study involving 9 Italian Rheumatology Units. Methods Data from consecutive 580 SLE, 354 pSS and 524 SSc patients were collected. Disease-specific features were registered in patients with known CD. Remaining patients were tested for IgA transglutaminase (Eu-tTG® human IgA new, Eurospital S.p.A., Trieste). Anti-endomysium (EMA) IgA and IgG were tested in IgA tTG positive and borderline patients. Esophagogastroduodenoscopy with duodenal biopsy was proposed in IgA tTG+/EMA+, IgA tTG-/EMA+ and IgA tTG±/EMA+ patients. Results CD prevalence was 1,7% in SLE, 7% in pSS and 1,3% in SSc patients. Higher frequency of elevated liver enzymes was detected in SLE-CD and of herpetiform dermatitis in SSc-CD patients in comparison to the other groups (p Conclusions The results of the present large multicenter study confirm higher prevalence of CD in CTD patients, especially in pSS. Screening of CD may be considered in younger patients with CTD and lower age at diagnosis. The strong association of CD with the diffuse type of SSc is of note and suggests that different, still unexplored, pathogenic mechanisms may characterize the two subsets of the disease. Disclosure of Interest None declared

Background Ultrasound pleural irregularity (PI-US) is a novel promising tool for non-invasive diagnosis of interstitial lung involvement (ILD) in connective tissue diseases (CTDs). Few data are available on its diagnostic usefulness in primary Sjogren9s syndrome (pSS)-induced ILD. Objectives a) To assess the accuracy of PI-US to diagnose ILD in pSS when compared to chest tomography (HRCT) (i.e gold standard imaging technique); b) to explore PI-US diagnostic value in early preclinical phases of lung involvement. Methods PI-US was performed by a single operator using a MyLab-25 (Esaote), 10 MHz, 5 cm linear probe. PI was defined as the loss of the normal hyperechoic linear pleural contour (score 0–2: normal, minimal and major changes at each intercostal space). Abnormal findings at HRCT were quantified by an expert radiologist according to a semiquantitative score (0–2: absent, moderate, severe). Semi-quantitative scores assigned by PI-US and HRCT to 6 lung fields (2 for the anterior, 2 for postero-superior and 2 for postero-inferior chest surface) were compared. Total and partial scores (for each lung fields) were evaluated. For statistical analysis chi-square, Mann-Whitney test, R-Spearman, and ROC-curve analysis were used. Results Validation study phase (PI-US vs HRCT): To validate PI-US technique, we enrolled 32 pSS patients [M/F:5/27; median age (IQR): 67 yrs (51.5–71); median disease duration (IQR): 7 (4–11) yrs; anti-Ro/SSA (+) 78.1%]. To be included patients should have performed a HRCT evaluation within 6 months. Thirteen patients (41%) presented HRCT lesions suggestive for ILD. HRCT total scores and PI-US total scores were strongly correlated (r=0.744, p=0.000). Similarly, PI-US and HRCT partial scores related to the postero-inferior fields showed a strong correlation one to each other (r=0.780, p=0.000). ROC-curve analysis identified a total PI-US score of 28.5 (Youden index) as able to predict HRCT-diagnosis of ILD with a sensitivity (SE) of 84.6% and a specificity (SP) of 89.5%. Analogously, a postero-inferior PI-US score of 12.5 demonstrated a SE of 100% and a SP of 89.5% for the ILD diagnosis. Prospective study phase exploring the usefulness of PI-US for ILD early pre-clinical diagnosis: We included 24 consecutive pSS patients without overt respiratory symptoms [M/F:1/23; median age (IQR): 55 yrs (47–67); median disease duration (IQR): 4 yrs (1–12); anti-Ro/SSA (+) 60.9%]. Out of them, at the end of the diagnostic work -up, four new cases of HRCT-proven pSS-ILD were diagnosed. Their PI-US mean total score was significantly higher than that observed in non-ILD patients (48±18 vs 16±12, p=0.001) as well as their mean postero-inferior PI-US score (19±9 vs 6±5, p=0.003). The total PI-US and postero-inferior PI-US cut-off retrieved in the first part of the study (i.e. 28.5 and 12.5) allowed us to identify those patients with an HRCT-proven pSS-ILD with a SE of 75% and 100%, a SP of 95% and 89.5%, a PPV of 75% and 57% and a NPV of of 95% and 100%, respectively. Conclusions Even if preliminary, this study demonstrated a strong correlation between PI-US and HRCT in the detection of ILD-pSS also in asymptomatic patients, opening new perspectives for the early non-invasive screening of lung involvement in pSS. Disclosure of Interest None declared

Background Joint involvement is one of the main causes of chronic pain and disability in SLE patients (pts); despite arthritis in SLE is usually considered mild, joint erosions and deformities can be observed with significant impact on patient9s quality of life. Imaging techniques are more sensitive than joint count in detecting synovitis and early joint damage. Objectives This study was aimed at evaluating the progression of joint damage in SLE and at evaluating predictive factors for damage accrual Methods Consecutive SLE pts with active hand-wrists synovitis (detected by joint count and/or ultrasounds) were enrolled in this 5-years prospective observational study. Clinical assessments as well as joint ultrasound (US) and MRI were performed at baseline and after 5 years. Each patient underwent a non-dominant hand–wrist US examination using a Logiq 9 with a linear probe operating at 14 MHz. Synovitis was defined as the presence of synovial hypertrophy and/or the presence of power Doppler signal (PD). A non-dominant hand–wrist MRI study with a 0.3 T extremity dedicated machine to evaluate the presence of bone erosions (BE) and bone marrow edema (BME) was also performed. Coronal and axial T1-weighted gradient-echo images and coronal STIR images were acquired. The images acquired were scored according to the RAMRIS scoring system for RA by a trained radiologist unaware of the clinical picture and diagnosis. Results Fifty-seven pts were enrolled (female 91.2%, mean age 44±12.2 years, mean disease duration 15.9±9 years);43 (75.4%) completed the follow-up, 3 died (5.2%) and 11 (19.4%) were lost to follow-up. At baseline, 7 (12.3%)satisfied criteria for Jaccoud arthropathy (JA) and 7 (12.3%) had a recent onset arthritis ( Conclusions Arthritis in SLE can be persistent over time and progress to joint damage even in a short tem period despite treatment; normal joint count at physical examination but US findings of synovitis can be associated with erosion progression over time. The presence of PD at US and bone marrow edema at MRI are associated with a more severe damage progression. US and MRI can be a valid help for the clinician to identify patients at higher risk of severe damage to be treated with a more aggressive therapeutic strategy targeting arthritis. Disclosure of Interest None declared

Background The EULAR recommendations for vaccination in adult patients with autoimmune rheumatic diseases strongly recommend inactivated influenza vaccination.Insufficient data are available about safety and efficacy of meningococcal C vaccination.In 2015–2016,after an increased incidence of meningitidis C infections in our country,the health care system has promoted a free meningococcal vaccination campaign Objectives To evaluate the adherence to the EULAR recommendations for influenza vaccination and to the meningococcal C vaccination campaign in a cohort of patients with autoimmune rheumatic diseases and to assess their safety.The efficacy in term of immune response to meningococcal C vaccination has been also evaluated Methods Consecutive in- and out-patients seen at our unit from February to December 2016 were enrolled in the study.Using a questionnaire created ad hoc the following data were collected:the percentage of patients who underwent influenza and/or meningococcal C vaccinations in the previous 12 months,the occurrence of adverse events and of disease flares after vaccinations,according with the report from the patients and with the rheumatologist clinical evaluation.Seroconvertion rates in patients and healthy controls were assessed using ELISA kits for human anti-meningococcal ACWY IgG antibodies.Antibody titres were expressed in U/ml and according with kit reference value were classified in absent,low,medium and high titre Results 286 patients (91% female) (143 SLE, 68 RA,60 Scleroderma,11 Sjogren Syndrome,3 Behcet disease and 1 Dermatomyositis) were included in the analysis.The mean age at evaluation was 52.9±16.1 years,mean disease duration was 15.3±10 years.The 53.1% of patients was taking steroids,at an average dose of 4.2 mg of 6-metilprednisolone/day,134/286 (46.9%) patients were on immunosuppressive therapies,of which 49/134 (36.6%) on biologic agents.The 19.9% (57/286) of patients underwent influenza vaccinations and the 13.3% (38/286) meningococcal C vaccination,8 patients underwent both vaccinations.No disease flares were observed after vaccination;seven patients reported non-specific adverse events after influenza (fever,discomfort,nausea,arthralgia) and 2 patients after meningococcal C vaccination (fever,rash at the injection site,discomfort).Seroconversion after meningococcal vaccination was analysed in 27 patients and 9 healthy subjects,no statistically significant differences in terms of antibody response to meningococcal vaccination were observed between these two groups.Treatment (steroids and immunosuppressive drugs) did not influence antibody titres Conclusions These data highlight the poor adherence to international recommendations on influenza vaccination in patients with autoimmune rheumatic disease at our Unit.The adherence to the meningococcal C vaccination campaign conducted in our country in 2015–2016 was also low.Our data confirm the safety of these vaccination and show that the immune response elicited by meningococcal C vaccination is comparable to healthy controls and is not influence by therapy References van Assen S, Agmon-Levin N, Elkayam O, et al.EULAR recommendations for vaccination in adult patients with autoimmune inflammatory rheumatic diseases.Annals of the rheumatic diseases 2011; 70(3): 414–22. Disclosure of Interest None declared

Background Early detection and prediction of glandular damage in primary Sjogren9s syndrome (pSS) is of pivotal importance in patients9 stratification and treatment. Recently, ultrasonography (SGUS) has appeared as a promising tool for the assessment of salivary gland involvement in pSS. However, few studies have specifically explored its role in the early identification of salivary gland damage and in monitoring disease progression over the follow-up. Objectives a) to explore the contribution of SGUS in the early assessment of pSS-induced glandular damage and in monitoring damage accrual during the follow-up b) to identify predictive factors associated with the development and progression of sonographic changes in salivary glands. Methods An inception cohort of 54 pSS (AECG 2002) patients was included in this study at the diagnosis and prospectively followed after 6, 12 and 24 months. Demographic, clinical data and the ESSDAI of the patients were collected at each study visit as well as SGUS score that was calculated every time by the same radiologist. Sonographic assessment of glandular damage was performed by evaluating the size of the parotid and submandibular glands (normal/reduced) and the presence/absence of hyperechoic bands in more than 50% of the glands (i.e fibrosis). Descriptive statistics and logistic regression were used for the data analysis. Results We included 54 patients (51F:3 M) with a median duration of symptoms before diagnosis of 11 months (range 36–5 mo) and a median (IQR) ESSDAI at baseline of 5 (1–8). We found that at baseline 13 patients out of 54 (24.1%) already presented one element of damage in their SGUS evaluation, 14/54 (25.9%) more than one element of damage, whereas only 27/54 (50%) of patients did not present any sonographic sign suggestive for damage. In particular, hyperechoic bands were detected in the parotid glands of 12/54 (22%) patients and in the submandibular glands of 21/54 (38.9%); reduced parotid and submandibular gland size were described in 5/54 (9.3%) and in 10/54 (18.5%) of the cases, respectively. Predictive factors associated with sonographic salivary gland damage at the diagnosis were the ESSDAI at baseline (OR =1.13 [95% CI 1.0 to 1.27], p=0.04) and the positivity for Rheumatoid factor (RF) (OR =4.47 [95% CI 1.29 to 15], p=0.02). During the follow-up, 15/54 (27.8%) patients presented a progression of their salivary gland damage. We specifically observed a significant increase in the frequency of reduced parotid (9.3% vs 25.9%, p=0.004) and submandibular gland size (18.5% vs 33.3%, p=0.008) at the end of follow-up. Predictive factors for damage accrual during follow-up were: minor salivary gland focus score (OR =3.5 [95% CI 1.1 to 10.6], p=0.03) and the ESSDAI at baseline (OR =1.64, [95% CI 1.0 to 2.6]p=0.03). Conclusions Sonographic assessment of glandular damage apparently revealed a relative high frequency of already established signs of glandular damage in pSS patients at baseline, especially in patients with positive RF and higher ESSDAI. When routinely used, SGUS may accurately allowed to monitor damage accrual over the follow-up, ultimately contributing to a better clinical management of patients. Disclosure of Interest None declared

Background Growing interest exists on the role of markers of subclinical cardiovascular disease as independent predictors of cardiovascular events. Poor data are available on the role of these markers as prognostic factors for Behcet9s syndrome (BS). Objectives The primary aim was to explore Intima-Media-Thickness (IMT), mean arterial diameter and distensibility (DC) in a group of patients with BS, comparing these data with a healthy control group and a disease control group; the secondary aim was to correlate the vascular parameters with demographic/clinical profile. Methods Thirty BS patients (females:12;mean age±SD:43±10.5; mean disease duration±SD:13±5.8) fulfilling the ISG criteria were prospective enrolled. Demographic data, level of disease activity, frequency of smokers, hypertension, family history of cardiovascular risk factors, body mass index (BMI) and current therapies were analysed. For each subject, ultrasound B-mode image sequences of right common carotid arteries were acquired and analysed by an automatic system (Carotid Studio,Quipu) for the measurement of IMT and mean arterial diameter. In addition, carotid pulse pressure (PP) was estimated by tonometry and DC coefficient was obtained. The systolic and diastolic carotid diameters were automatically measured on the distal wall of the common carotid artery, 1–2 cm beneath the bifurcation. Carotid diameter was calculated as the distance between media-adventitia interfaces. Cross-sectional DC was estimated through the variations in arterial cross-sectional area and blood pressure during systole. DC was computed as DC=ΔA/(PP*A) where A is the diastolic lumen area, ΔA is the stroke change in lumen area, and PP is the local pulse pressure. Results At time of evaluation, 4/17 patients presented active disease (50% ocular involvement, 25% joint involvement, 25% gastro-enteric involvement; mean BS activity score 5). Mean IMT±SD value resulted of 0.57±0.81, mean arterial diameter±SD value was 6.879±0.81 and mean DC± SD 27.3±14.34. All the vascular parameters considered were significant correlated with BMI, while only IMT and DC were also significant correlated with arterial hypertension. Using a correction analysis for age and sex, we found significant correlations between mean arterial diameter and disease activity and between DC and disease duration. These data resulted significantly different compared to healthy control and a disease control groups, in terms of smaller arterial diameter and higher DC. Conclusions Our data have shown that there is a consistent influence of disease activity and duration of disease on mean arterial diameter and DC, respectively. Thus, it is desirable that future pharmacological researches on BS target on this issue. Acknowledgements Seyahi E, Ugurlu S, Cumali R, Balci H, Ozdemir O, Melikoglu M, Hatemi G, Fresko I, Hamuryudan V, Yurdakul S, Yazici H. Atherosclerosis in Behcet9s Syndrome. Semin Arthritis Rheum. 2008 Aug;38(1):1–12. Seyahi E, Ugurlu S, Cumali R, Balci H, Ozdemir O, Melikoglu M, Hatemi G,Fresko I, Hamuryudan V, Yurdakul S, Yazici H. Atherosclerosis in Behcet9sSyndrome. Semin Arthritis Rheum. 2008 Aug;38(1):1–12. Disclosure of Interest None declared

Background Belimumab is used to treat several systemic lupus erythematosus (SLE) manifestations and predictors of response are advisable in clinical practice. Objectives To explore the effects of belimumab treatment on skin involvement in SLE patients in a real-life setting. Methods SLE patients treated with belimumab (10 mg/kg day 0, 14, 28 and then every 28 days), as add-on therapy in 11 Italian cohorts were prospectively followed-up for 24 months. Skin involvement was measured by the CLASIa score (Cutaneous Cutaneous LE Disease Area and Severity Index Activity) at baseline and every six months until month 24. Damage was measured by CLASI-Damage (CLASId). Response was defined as CLASIa The following variables were tested to determine baseline predictors of response at 12, 18 and 24 months: gender, age, disease duration, SLE activity index 2000 (SLEDAI-2K) ≥10, prednisone dose >7.5 mg/day, baseline CLASIa and CLASId, concomitant immunesuppressants (yes/no) and number of previous immunesuppressants. Statistical analysis was performed with SPSS 22.0 software. Results 188 patients were studied, among whom 48 (25.5%) had skin involvement as the leading feature for belimumab therapy. Mean follow-up period was 17.5±10.96 months. Thirty-eight patients completed a 6-month follow-up; 27 completed a 12-month follow-up; 19 completed a 18-month follow-up and 15 completed a 24-month follow-up. Fourteen patients discontinued due to adverse events (7/14, 50%), lack of efficacy (4/14, 28.5%) or other causes (3/14, 21.4%). CLASIa, daily prednisone dosage and SLEDAI-2K significantly decreased during the follow-up (p Conclusions Belimumab use in cutaneous involvement is associated with reduced activity in skin lesions and hindrance of skin damage. Early use of belimumab before damage is established is likely to be associated with a better response. Acknowledgements dr. Margherita Zen, dr. Maddalena Larosa, dr. Francesca Ometto, dr. Mara Felicetti for helping in data collection and statistical analysis Disclosure of Interest None declared

Background EULAR and ACR are supporting multi-phase development of SLE classification criteria based on weighted criteria and a continuous probability scale. Prior steps included criteria generation, criteria reduction through Delphi and Nominal Group Technique exercises, literature review for sensitivity/specificity of candidate criteria, and organization of candidate criteria into seven clinical and three immunologic domains. Objectives To refine definitions of candidate criteria, determine relative weights using multicriteria decision analysis, and determine a threshold score for SLE classification. Methods An SLE Expert Panel (9 North American, 8 European) submitted 167 unique cases with a range of SLE probability. Experts scored 20 representative cases using the candidate criteria and rank-ordered them. In a 2-day meeting, experts reviewed inter-rater reliability of scoring, refined criteria definitions, and participated in a multicriteria decision analysis (MCDA) exercise using 1000Minds TM software. Experts were presented a series of decisions between two cases, each with different criteria from two domains (e.g. oral ulcers [cutaneous] and acute pericarditis [serositis] vs. alopecia [cutaneous] and pleural effusion [serositis]) and anonymously voted for the case more likely to be classified as SLE. Votes were discussed until consensus was reached for each decision. Using the consensus decisions, 1000Minds™ calculated criteria weights, assigned a total score to each of remaining 147 cases and rank-ordered the cases. Experts voted on whether each case should be classified as SLE. MCDA was repeated for criteria whose calculated weights were inconsistent with expert opinion until group consensus was achieved. 1000Minds TM then re-calculated criteria weights and re-ranked cases once. The score of the last case for which expert consensus was achieved was the threshold score. Results Inter-rater reliability was good; human data entry error, not following instructions, and differing interpretations of criteria definitions accounted for discrepancies. Arthritis and pericarditis definitions were modified through group discussion. The MCDA involved 74 pairwise decisions. Cranial neuropathy and Class VI lupus nephritis were removed as they added little to SLE classification. MCDA was repeated for the arthritis and cutaneous domains as initial weights did not match expert opinion. After criteria weights and scores were re-calculated once, experts reached consensus for SLE classification for case score >83. Conclusions Using an iterative process, the expert panel refined definitions, weighted candidate criteria and determined a threshold score of >83 for SLE classification, which will undergo validation. Acknowledgements Joint support from EULAR and ACR Disclosure of Interest None declared

Background Evaluation of disease activity is one of the most important assessments in SLE; several instruments have been developed based on clinical and laboratory information recorded by the physician. Patient-administered questionnaires provide useful information with significant time and cost saving. Indeed, patient-reported outcomes are gaining a central role as outcome measures.SLAQ is a patient-reported instrument for the assessment of disease activity in SLE. It consists of three scores: Patient Global Assessment question (PGA) about presence and severity of lupus activity over the past month, questions on 24 symptoms (SLAQscore) and a single Numerical Rating Scale (NRS) for disease activity (0 – 10). Objectives The aim of the study was to translate and to validate the SLAQ in Italian. Methods The process of translation and cultural adaptation followed published guidelines (1). The final version of the questionnaire (SLAQit) was pretested in a group of 35 SLE patients to assess acceptability, comprehension and feasibility. The validity of the SLAQit was evaluated by its administering to consecutive SLE patients attending the outpatient9s clinic or the inpatients wards. Internal consistency between the three components of the score was evaluated by Chronbach9s alpha; the external validity was tested toward validated activity indices (SLEDAI and ECLAM) scored by a physician blinded to the SLAQ results. In a subgroup of 30 patients the questionnaire was administered twice at 2 weeks9 interval to assess its reliability. Results 137 patients were enrolled (92% female, mean age 43.1 years, mean disease duration 15.3 years). At enrollment, the median SLEDAI score was 2 (range 1–18) and 45% of patients had at least one organ damage (median 2, 1–8). The pilot test provided a good acceptability (99.9% of response rate) and feasibility (mean of 4.6±2.3 minutes to be completed, 1–10); moreover, the 100% of patients declared to comprehend the scope of the SLAQ and 67.5% declared no content comprehension problems. Internal consistency was very good between (NRS vs PGA vs SLAQscore (α=0.79). NRS and PGA showed a linear correlation with both ECLAM and SLEDAI scores (ρ=0.24, p=0.004 and ρ=0.45, p 0.8 for NRS, PGA and SLAQscore). SLAQit scores resulted directly related to the patients age (p=0.002) and the SLICC score (p=0.003) while no correlation with disease duration was observed. Conclusions SLAQit demonstrated to be acceptable, comprehensible and feasible in our routine clinical setting; it also showed good internal consistency; correlation with physician9s driven instruments is weaker and the SLAQit was influenced by epidemiological and disease-related factors (i.e. damage), thus confirming that the disease perception from the patient9s perspective can be different from physicians and influenced by several factors. The SLAQit can be considered a useful screening tool for the first assessment of the disease activity before the standard visit. References Guillemin F, et al. Journal of Clinical Epidemiology 1993; 46: 1417–1432. Disclosure of Interest None declared

Background Dry nose is reported quite frequently by patients affected by primary Sjogren9s syndrome (pSS) in daily practice. However, a clear definition of nasal involvement in pSS is not available. Objectives a) to explore clinical presentation of nasal involvement in pSS analyzing key symptoms, objective findings at the inspection of the external and inner nose and nasal cytology b) to investigate any associations/correlation between nasal involvement and other clinical-serological disease manifestations c) to assess the overall impact of nasal involvement on patients reported outcomes (PROs). Methods Consecutive pSS patients (AECG 2002) were seen by a team of rheumatologists and ENT specialists. In addition to a standard rheumatologic evaluation, all the patients underwent a complete ENT evaluation. Nasal symptoms (i.e dry nose, nasal stuffiness, crusting, hyposmia) were collected by an “ad hoc questionnaire” and scored by the patients on visual analogique scales. Inspection of the external and inner nose, endoscopy of the nasal cavity and nasal cytology were performed as well. Allergy testing were also carried out when indicated. The following tools were used to assess PROs: ESSPRI, SF-36 and SNOT-22. Results Forty-six pSS patients were included in the study [M:F =45:1; median age (IQR):64 (53–70); median disease duration (IQR): 66 months (24–120)]. Nasal symptoms ranged from: dryness in the nose (56.5%), crusting 10/46 (21.7%), nasal stuffiness 20/46 (45.5%) and hyposmia 7/46 (15.2%). Thirteen patients did not present signs of nasal involvement, whereas 21 patients (45.7%) presented rhinitis sicca (RS), 6 (13%) allergic rhinitis (AR), 4 (8.7%) chronic rhinosinusitis (CRS) and 2 (4.3%) non-allergic rhinitis (NAR). Patients with nasal involvement were more frequently seronegative (p=0.04) and presented significantly higher SNOT-22 scores (p=0.008) when compared to patients without nasal involvement; no additional demographic or clinical differences between the two groups. Allergy testing were more frequently positive in patients with RS and AR. Nasal cytology showed that the rates of the cells (eosinophils and neutrophils) in patients without nasal involvement were negligible whereas they were significantly increasead in pSS patients with RS and AR. The SNOT-22 (r=-.433, p=0.02) and the scores assigned to the VAS of nasal dryness (r=-.755, p=0.003) and crusting (r=-.794, p=0.001) strongly correlated with SF-36 questionnaire. SNOT-22 also correlated with the ESSPRI (r=.399, p=0.04), whereas we did not find a correlation between VAS scores assigned to nasal dryness and VAS scores of oral and ocular dryness. PROs related to nasal symptoms were significantly influenced by a concomitant diagnosis of fibromyalgia. Conclusions Rhinitis sicca was the most common clinical presentation of nasal involvement in pSS patients, especially in seronegative patients. Apparently, nasal symptoms correlated weakly with ocular and oral dryness. PROs exploring nasal symptoms revealed that nasal involvement impact significantly on patients quality of life. Disclosure of Interest None declared

Background The development of microchimerism after solid organ transplantation, but also after blood transfusions and during pregnancy, is no longer considered controversial. Controversy still exists on the immunological significance of this phenomenon, such for example its role on the development of tolerance to the graft, or of some autoimmune conditions such as scleroderma (SSc). In this respect several studies have showed the presence of fetal cells not only in the peripheral blood but also in the skin of SSc patients. These observations, along with epidemiological (female prevalence, onset after childbearing years) and clinical (similarities with chronic graft versus host disease) data, have led to the hypothesis that microchimerism may play a role in the aetiopathogenesis of this disease. Objectives The aim of our study was to evaluate the prevalence of microchimerism in a population of SLE patients in comparison with SSc patients followed at our Unit. Methods Anticoagulated peripheral blood was obtained from 22 female patients (13 SLE and 9 SSc) who had a previous male pregnancy followed at our Unit. Genomic DNA was isolated from anticoagulated blood using a DNA extraction kit. A specific Y-chromosome sequence was detected by amplifying DNA in a nested-polymerase chain reaction (PCR) using published primers. Results Microchimerism was present in 62% of SLE patients and 89% of patients with SSc, the mean time interval between the pregnancy and the analysis was of 27 years and no significative differences were observed between positive and negative patients. In all scleroderma patients the pregnancy occurred before the onset of the disease (onset of Raynaud’s phenomenon). In SLE patients 38% of the pregnancies occurred before the onset of the disease, and 62% after the onset of the disease, however no differences in the development of microchimerism were observed. Conclusion Since the literature data report a prevalence of 33% of microchimerism in healthy controls, these preliminary data may suggest that microchimerism develops more easily in patients with various autoimmune conditions. Further studies on extended populations of CTD patients and healthy controls appear necessary, in order to look for permissive factors for its development, such for example immunosuppressive therapy or immunological abnormalities. References Evans PC, et al. Blood 1999;93:2033–7 Nelson JL. Lupus 1999;8:370–4 Nelson JL. Curr Opin Rheumatol. 1998;10:564–71

Background Diffuse Proliferative Glomerulonephritis (DPGN) is considered one of the most important factors influencing the long term prognosis of SLE patients. Renal flares are considered important risk factors influencing the outcome of renal involvement. Objectives The aim of this study was to evaluate the occurrence of renal flares and their prognostic significance for the renal outcome in a population of SLE patients with DPGN. Methods The clinical, laboratory and histological data of 91 SLE patients (8 males and 83 females) followed at the Rheumatology Unit of the University of Pisa, were retrospectively evaluated. Results Fourty-nine patients (54%) presented a renal flare a mean of 42 months after the renal biopsy. Twenty-eight flares (57%) were proteinuric, twenty-one (43%) were nephritic flares. A young age at the time of the renal biopsy and treatment with steroids alone, were significantly correlated with the occurrence of renal flares, furthermore a high Activity Index (AI >9) and the presence of karyorrhexis on renal histology were correlated with the occurrence of nephritic flares. A poor renal outcome occurred in 27% of the patients; serum creatinine levels at the time of renal biopsy, the presence of karyorrhexis and the composite Chronicity Index (CI) as well as all of its component on renal histology, the number of renal flares (both proteinuric and nephritic), the occurrence of nephritic flares and early proteinuric flares (i.e. in the first 17 months after the renal biopsy), were correlated with a poor renal outcome. Conclusion In conclusion, the analysis of our patients has showed that nephritic flares and early proteinuric flares are important risk factors for the occurrence of a poor renal outcome. Since young patients with an elevated AI and karyorrhexis are at increased risk for renal flares, they should be candidated for a more aggressive and prolonged treatment. References Moroni G, et al. Kidney Int. 1996;50:2047–53 Ponticelli C, et al. Lupus 1998;7:635–8 Ciruelo, et al. Arthritis Rheum. 1996;39:2028–34