Objectives:Inflammatory rheumatic and musculoskeletal diseases (RMDs) are hallmarked by an increased risk of cardiovascular (CV) disease compared with the general population. Evidence suggests that CV risk remains under-recognised and underdiscussed in routine care, although data on patient experience is lacking. We aimed to assess awareness, perceptions, and experiences regarding CV risk and prevention in patients with RMDs. Methods:An anonymous, online survey was codesigned by rheumatologists, researchers, and patient research partners in English language and translated into 9 languages. This was distributed through patient organisations/forums and social media. Results:A total of 951 patients with RMDs from 43 countries (mainly females, aged 50-70 years, and with a diagnosis of rheumatoid arthritis, spondyloarthritis, or systemic lupus erythematosus) completed the survey. Although 68% of respondents acknowledged an increased CV risk in RMDs, and traditional CV risk factors were recognised by up to 60% of the participants, knowledge of disease-specific contributors (eg, disease activity) to CV risk was limited. Only 35% of respondents had ever discussed CV risk with a healthcare provider, mainly rheumatologists or cardiologists, and the content primarily focused on lifestyle advice. Lack of awareness on the topic relevance (38%), perceived complexity of the topic (25%), limited time of clinical visits (35%), and lack of informative materials (33%) emerged as the main key barriers among those poorly/not informed. Conclusions:This large, multinational survey demonstrates substantial gaps in CV risk awareness, knowledge, and patient-provider communication among patients with RMDs. These figures support the development of structured, patient-centred educational strategies and enhanced multidisciplinary communication schemes to improve CV prevention in RMDs.

OBJECTIVES:Patient education is increasingly acknowledged as an important aspect of the management of systemic lupus erythematosus (SLE). The aim of the study was to develop the SLE Knowledge Assessment score (SLAKE), a digital multilingual self-assessment tool designed to quantify essential SLE knowledge. METHODS:International healthcare professionals (HCPs) and patient representatives engaged in a multi-step process to: identify essential SLE knowledge domains, select key domains via rating, and generate an item bank of 394 questions across 11 domains, which was then adapted into 19 languages. For validation, participants completed 44 questions (including 33 randomly selected), with scores calculated for total knowledge and the 11 specific domains. Statistical analyses examined associations between scores and demographic, clinical, and educational variables. RESULTS:SLAKE was used by 1182 SLE participants (1120 [94.8%] women, median age: 45 years [IQR: 35-54 years]), with a median SLE duration of 10 years (IQR: 4-20 years). The median SLAKE score was 37 (IQR: 34-40) of a maximum of 44 points while the median score across the 11 SLAKE domains ranged between 3 and 4 over a maximum of 4 points. There was a significant positive association between SLAKE score and SLE duration (p= 0.006), previous participation to a patient education course or a patient training for lupus (p< 0.0001) and the education level (p< 0.0001) but not with age (p= 0.48) or gender (p= 0.39). CONCLUSION:SLAKE is a valid, multilingual, digital self-assessment tool that effectively measures essential SLE knowledge. Its randomized question bank and domain-specific scoring enable targeted education, ultimately supporting better disease management.

OBJECTIVES:To develop and validate a European Alliance of Associations for Rheumatology (EULAR) disease activity score in antiphospholipid syndrome (EAPSDAS). METHODS:Twenty-four Task Force members and an international group of 53 antiphospholipid syndrome (APS) experts/collaborators, 65 patients with primary APS, and 21 healthcare professionals participated. EAPSDAS development proceeded in 4 phases: (i) item generation using a systematic literature review and 2 surveys; (ii) item reduction by rating items on their importance to be included in EAPSDAS and using Delphi methodology; (iii) item scoring based on real-world clinical vignettes and using as criterion standard the physician global assessment (PhysGA); and (iv) validation. RESULTS:One hundred seventy items representing APS activity were generated, and 140 deduplicated candidate items were rated by participants. Using a ≥75% vote threshold and Delphi consensus among Task Force members, 24 items were included in the EAPSDAS thrombotic/microvascular/nonthrombotic (TMN) scale and 6 in the obstetric scale. Item scoring was based on ratings of 3 versions of 60 vignettes with new/worsening manifestations (30 single TMN or obstetric manifestations, 26 combinations of 2 TMN manifestations, 2 inactive cases, 2 testing cases) by physicians. Item scores were calculated as the adjusted mean PhysGA in linear regression analysis. A 1-month time frame was defined for the TMN scale and the entire pregnancy for the obstetric scale. Scores for stable or improved TMN manifestations were also included. High face and content validity, construct validity (internal/external standard), and reliability were demonstrated using real-world clinical vignettes. CONCLUSIONS:Using data-driven and consensus methodology, EAPSDAS was developed and initial validation was performed. Further validation in prospective studies is warranted.

Background: Counselling patients with rheumatic and musculoskeletal diseases (RMD) in the phase of reproduction, pregnancy and lactation is a challenging task. In women, the potential risk of drug therapy needs to be weighed against the risk of untreated maternal RMD and associated risks for the mother and the child. In men, the safety of antirheumatic drugs with regard to fertility and pregnancy/offspring outcomes needs to be considered. The first EULAR points to consider (PtC) for the use of antirheumatic drugs before pregnancy, and during pregnancy and lactation published in 2016 have been widely used [1]. Meanwhile, modern treatment approaches have evolved towards a treat-to-target concept to avoid the negative impact of active disease on reproductive function and pregnancy outcomes. Additionally, several relevant data have emerged about antirheumatic drugs in reproduction, pregnancy and lactation. Objectives: To update the EULAR PtC for use of antirheumatic drugs in reproduction, pregnancy and lactation including additional drugs and adverse outcomes as well as paternal drug safety. Methods: According to the EULAR standardised operating procedures, the task force involving 27 international members formed six PIO research questions for the systematic literature review (SLR) (detailed protocol registered in PROSPERO, No CRD42022357689). The result of the SLR was presented to the task force during a consensus meeting in October 2023 to develop this update. A predefined voting process was applied to each overarching principle and statement. Level of evidence and strength of recommendation were assigned, and participants finally provided their level of agreement to each item. Results: The SLR yielded 8025 articles (2015-2023: 5183; 2000-2015 for outcomes not covered in previous EULAR PtC: 2842), of which 2385 full text articles were reviewed for eligibility. Out of those, 307 were extracted and further analysed. Based on the result of the SLR, the task force developed 5 overarching principles (Table 1), 12 PtC for the use of antirheumatic drugs before and during pregnancy, 4 PtC for the use of antirheumatic drugs in lactation and 3 for the use of antirheumatic drugs in male patients (Table 2). The current evidence indicates that synthetic DMARDs compatible with pregnancy include azathioprine or mercaptopurine, chloroquine, colchicine, cyclosporine, hydroxychloroquine, sulfasalazine, and tacrolimus. Regarding NSAIDs and glucocorticoids, a more restrictive approach to their use during pregnancy is recommended. Additionally, due to a more favourable risk–benefit profile, the use of bDMARDs, including nonTNFi, is more permissive. In relation to lactation, compatible drugs include azathioprine or mercaptopurine, celecoxib, chloroquine, colchicine, cyclosporine, hydroxychloroquine, IVIG, methylprednisolone pulses, non-selective NSAIDs (e.g., ibuprofen), prednisone and prednisolone, sulfasalazine, and tacrolimus. A more permissive use is recommended for bDMARDs. Finally, concerning the use of drugs in men, compatible options include azathioprine or mercaptopurine, colchicine, cyclosporine, hydroxychloroquine and chloroquine, IVIG, leflunomide, methotrexate (≤25 mg/week), mycophenolate, NSAIDs, prednisone and prednisolone, sildenafil, sulfasalazine, tacrolimus, and bDMARDs. Recommendations on infant vaccinations are provided. Conclusion: The updated EULAR PtC provide consensus guidance and will help to improve the management of patients during the phase of reproduction, pregnancy and lactation. REFERENCES: [1] Gotestam Skorpen, 2016, DOI: 10.1136/annrheumdis-2015-208840. Table 1. EULAR overarching principles for the use of antirheumatic drugs in reproduction, pregnancy and lactation Table 2. EULAR points to consider for use of antirheumatic drugs in reproduction, pregnancy and lactation Acknowledgements: NIL. Disclosure of Interests: Frauke Förger Mepha, Roche, UCB Pharma, MSD, UCB Pharma, GSK, Andrea Pluma Sanjurjo: None declared, Linda Rüegg: None declared, Sabrina Hamroun: None declared, Irene Cecchi: None declared, Luis Fernando Perez: None declared, Philip Anderson: None declared, Laura Andreoli: None declared, Sara Badreh: None declared, Vladimira Boyadzhieva: None declared, Christina Chambers: None declared, Nathalie Costedoat-Chalumeau: None declared, Radboud J.E.M. Dolhain: None declared, Rebecca Fischer-Betz: None declared, Ian Giles: None declared, Carina Götestam Skorpen: None declared, Maria Hoeltzenbein: None declared, Francesca Marchiori: None declared, Karoline Mayer-Pickel: None declared, Anna Moltó: None declared, Catherine Nelson-Piercy: None declared, Ole Haagen Nielsen: None declared, Angela Tincani: None declared, Marianne Wallenius: None declared, Astrid Zbinden: None declared, Yvette Meissner Lilly, Pfizer, Axel Finckh: None declared.

Background Systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) have a high disease burden which cannot be sufficiently alleviated with pharmacological treatment alone. In summer 2023 EULAR recommendations for non-pharmacological management of SLE and SSc were published https://doi.org/10.1136/ard-2023-224416. Dissemination and implementation of the recommendations into daily clinical practice is crucial due to the impact on patients QoL and on a holistic management of these diseases. Methods We first aim at raising awareness of the recommendations to ensure adoption in clinical practice. Twelve European national teams consisting of patient representatives, Health professionals (HPRs), and physicians engaging in the care of people with SLE and SSc have been formed. Each national team will disseminate the recommendations, e.g., in the form of translations, publications, mailings, presentations (step 1). An online survey (step 2) will be prepared, translated, checked by native speakers, and pilot-tested in a smaller group of patients. The survey gauges both the professional and experiential opinion of stakeholders' perceived alignment of each recommendation with current clinical practice in their respective countries. The survey will be circulated to SLE and SSc patients, HPRs, and physicians. Qualitative data will be translated, checked by native speakers, and analysed using qualitative methodology. Project reports results including the result of the analysis will be published both at national, and global level (step 3). The role of patient representative is key in all the steps. Results The first twelve national teams were formed in Switzerland, Austria, Belgium, Germany, Greece, Hungary, Italy, Portugal, Sweden, United Kingdom, Denmark, the Netherlands. The congress is the occasion for discussing the EULAR recommendations, showing the first results of the national teams disseminating the recommendations and inviting stakeholders from other countries to participate in the project and in its next steps. Conclusions This project aims to provide a foundation for disseminating and implementing the recent published EULAR recommendations for the non-pharmacological management of SLE and SSc. All stakeholder groups involved can use their network to disseminate the recommendations and amplify the reach of the recommendations in a multi-tiered approach. In this action research, the role of patient representatives is key.

Background: In the absence of strategic direction, patient groups struggle to deliver results that meet their ambitions. Projects selection based on short term considerations or association to funding combined with changing priorities prevent more significant projects implementation. Objectives: To share Lupus Europe experience on how a simple strategic plan helped achieve long term goals and mobilise resources. Methods: Back in 2011, Lupus Europe was a small organisation, with less than 30k€ annual budget, and one key event per year. The Board then decided to take a more strategic approach. Starting from defining a simple and clear vision ("A fulfilling life for all people living with lupus in Europe, until we reach a world without lupus"), the organisation defined the biggest contributions it could make to reach it, which became 4 Strategic objectives (Research is supported, Members are Empowered and energized, Selected partners and media make lupus voice heard and the community is sustainable). From these cascaded a choice of ways to achieve them (our strategic pillars) for each of which we defined a number of 5 years measurable deliverables to pursue. These were cut in intermediate steps, leading to a 5 years plan, aligned with clinicians and academics. The plan was then voted by our members and presented to our partners, organised in visual ways that help memorise it and structure our presentations. It became the backbone of our communication, decision making, actions and reporting: At each Board meeting, we review progress on our annual steps, adjusting agendas and activities to meet the goals and assigning leadership where needed. Around September, we look at the next years' steps and amend them if needed to better reach our 5-years's goals and prepare our funding requests to industry, based on OUR projects and plans, not theirs. Results: In 2023, Lupus Europe is in its 3rd "5-years plan". The number of industry sponsors grew from 2 to 14. We are recognised as an EMA partner. Our volunteers network delivered more than 3,000 hours of support from more than 50 motivated volunteers. We run patient panels, surveys, a Patient advisory network, consistent social media and a website translated in 15 languages. We can approach partners with clear asks and select the projects we think add real value, and these projects are incredibly bigger than what we imagined ever reaching 12 years ago. Our total budget now reaches 400k€, more than we need for all our planned activities, allowing us to reject any sponsor not meeting our requirements. We measure our progress 3 times a year, allowing corrective action where needed. Our plan allows us to focus on priority and reject distractions. Virtually all our members and industry partners know our strategies, repeated year after year as the anchor of all we do, and can explain how they fit together. We were rated "best in class" by many industry partners on our "professional strategic focus" and the quality and quantity we deliver as a volunteer based organisation. We know where we go, and this reinforces our sense of purpose, our energy to deliver, our confidence and our feeling great as a team. Conclusion: Designing a Strategic plan is a key enabler that all patient organisations, regardless how small they are, can implement. It guides actions and helps achieve objectives. It is substantially easier to do than many think, but requires patience to install. REFERENCES: NIL. Acknowledgements: None of the authors has direct conflict of Interest. However, LUPUS EUROPE is funded mostly by grants or donations from Pharmaceutical Companies (Astra Zeneca, Biogen, BMS, Boehringer-Ingelheim, Galapagos, GSK, Idorsia, Janssen, Merck, Novartis, Otsuka, Roche, UCB), none of which exceeds 20% of total funds collected, and none having a say on the content of our studies. Disclosure of Interests: None declared.

Background: At EULAR 2023, the Lupus100 initiative was launched to address the issue of unreliable online sources by ensuring 95% of European lupus patients can access high-quality information in their native languages. This was made possible thanks to the effective collaboration between patients and doctors who worked together to create already 13 different language versions, available online. Reflecting on the first year since Lupus100's launch can provide valuable insights into patient engagement and information dissemination. Objectives: To analyse the most frequently consulted questions on the Lupus100 website to gauge patient concerns, pinpoint significant information needs, and identify any prevalent misconceptions. Methods: Google Analytics, user metrics such as page views, unique user counts, and content specifics were tracked from October 2022 to January 2024 to identify the resulting most commonly viewed questions among users and identify any trends. Results: The English site has attracted 151,225 views by 54,959 unique users. The multilingual websites have been similarly impactful. The top 10 questions looked at in detail were as follows: 1.What is lupus? 2.Is lupus contagious? 3.What are the different forms of lupus? What is drug induced lupus? 4.Can I have a normal sex life? 5.Is there a relationship between lupus and stress? 6.Why does lupus mainly affect women? Does male lupus exist? 7.What is neonatal lupus? 8.Can lupus be associated with other autoimmune diseases? 9.Should we follow a special diet in lupus? 10.I have lupus. I also have symptoms of Sjogren's syndrome or antiphospholipid syndrome. Do I really have several diseases? The navigation analysis, using "heat maps" and mouse movements also revealed that a significant proportion of users focused on the short answers provided under each question rather than the longer explanatory texts giving additional background and deeper explanation. This data highlights the necessity for patient associations to tailor information that is both foundational (what is lupus, is it contagious) and in-depth (diet, association with other diseases,..), ensuring that patients can find answers to their most pressing questions. High user engagement with quick answers rather than full explanations also suggests a need for more accessible, concise information on lupus. Conclusion: The data from Lupus100.org allows for the shaping of therapeutic patient education based on the actual questions that patients have. Continual monitoring of the Lupus100 site will help gain further insights to tailor communication efforts to the evolving needs of the community. REFERENCES: NIL. Disclosure of Interests: None of the authors has direct conflict of Interest. However, LUPUS EUROPE is funded mostly by grants or donations from Pharmaceutical Companies (Astra Zeneca, Biogen, BMS, Boehringer-Ingelheim, Galapagos, GSK, Idorsia, Janssen, Merck, Novartis, Otsuka, Roche, UCB), none of which exceeds 20% of total funds collected, and none having a say on the content of our studies. Acknowledgements: NIL. Disclosure of Interests: None declared.

Background Despite significant improvements in diagnosis delay and treatment strategies, the burden of Systemic Lupus Erythematosus (SLE) remains high. Objectives The objective of the study was to assess the association between diagnosis delay, disease activity and burden on daily life (BoDL) in a large sample of European patients with SLE. Methods In May 2020, Lupus Europe, the European umbrella patient association for SLE, conducted a multilingual anonymous online cross-sectional study to individuals with a self-reported physician’s diagnosis of SLE living in Europe. The BoDL score was computed using 1 to 5 Likert scales on 5 domains (mobility, anxiety/depression, self-care, daily activities and pain/discomfort) and the sum was transposed on a 0 (minimum Burden on daily life) to 100 (maximum BoDL) scale. Comparisons between independent groups were made using the Mann-Whitney test for continuous outcomes and the Chi-2 test (or Fisher’s exact test) for quantitative data. Results Data of 4,150 SLE patients from 35 European countries were analysed. The mean (±SD) BoDL score in the study population was 37.8 (±18.7) with a modest downward trend of the BoDL based on age (from 33.4% to 42.1% from age less than 25 to age 65 - a loss of up to 9% points over up to 40 years). The diagnosis delay was reported to be <2 years in 1903 participants (47.5%), between 2 and <5 years in 1056 (26.3%) and ≥5 years or more in 1049 (26.2%). 142 did not answer.. Those with a diagnosis of SLE within 2 years of first symptoms had significantly lower mean Burden on daily life scores than those diagnosed after 5 years (33.6 versus 44.0, p<0.001). This trend is deemed robust as it was found across almost all European countries. These results highlight the importance of improving current diagnosis delay for SLE as a way to improve the burden of the disease on daily life. A total of 2980 (71.8%) patients felt that their “lupus has been under control over the last 3 months” while 1166 (28.1%) did not. 4 did not answer. The Burden on daily life score was significantly better in SLE patients feeling that their lupus had been under control during the past 3 months versus the others (34.0% versus 47.6%, p<0.001). Again, this trend was found across almost all European countries. Conclusion This large patient survey reveals both the importance of prompt SLE diagnosis as well the relationship between disease activity and disease burden upon the daily life of European lupus patients. Further improvements should focus on reducing the diagnosis delay and identifying new therapeutic strategies for those with uncontrolled disease. Healthcare pathways, which may accelerate diagnosis and optimize therapeutic management, are necessary to improve patients’ outcomes in SLE. Reference [1]Cornet A, Andersen J, Myllys K, et al Living with systemic lupus erythematosus in 2020: a European patient survey Lupus Science & Medicine 2021;8:e000469. doi: 10.1136/lupus-2020-000469 Acknowledgements: NIL. Disclosure of Interests None Declared.

Background The Patient – Doctor communication gap is often discussed, and is one of the obstacles pointed by patients and doctors alike in the fruitful co-working of optimal treatments. Recognizing the difference between doctors and patients approach when confronted to a lupus flare is critical to bridge this gap and increase the effectiveness of jointly agreed treatment plans. Objectives Identify potential discrepancies between patient and doctors discussion starting points when facing a lupus flare. Are they “talking the same thing?”, “do they have similar concerns in their minds?” Methods As part of the SL Euro lupus 2022 congress 57 participants, doctors and patients, took part in a speed-workshop to identify first “what makes them identify an event as a lupus flare” and then their “key concerns when facing a lupus flare”. Answers were provided on post-its of different colours per type of participants, forcing short answers focusing on key aspects. The commonalities and discrepancies where then identified for further handling. Results A total of 57 statements identifying a lupus flare were collected and classified assessable (visible/ measurable) items, or specific symptoms. The results highlighted that the communication difficulty between patients and doctors starts from the very feeling of what a flare is. 91% of patients include in their recognition of a flare a factor that cannot be “easily” objectivated by a doctor such as fatigue (59.1%), or pain (50%). Only 36% of patients include an externally visible factors, most often fever. In contrast, 88% of doctors require an externally visible/measurable factor to “identify” a flare. These 2 different starting points can create a first communication gap, as well as a difference in the qualification of an event as a flare or not.A total of 93 concerns, sorted in “huge”, “big”, or ”mid” were collected from participants. These items could be classified in 4 key groups: concerns around daily life & logistics; overall anxiety; symptoms and medication. When confronted to an event considered as a flare, patients have a perspective focusing on the impact of flares on their daily life and logistics (35% of huge concerns, 30% of all concerns), and face anxiety over disease/life in the future (30% of all concerns 19% of huge concerns); on the contrary the doctor's focus is on symptoms (67% of huge concerns, 46% of all concerns) and medications (29% of all concerns), with only 7% of the doctors focusing on the patients “immediate” daily life/logistics issues, and 18% on anxiety. This discrepancy of concern also hinders the patient-doctor communication. Conclusion Doctors and Patients starting points and concerns with regards to a lupus flare appear perfectly logical and fitting each individual's role in the relationship, with the doctor's primarily focused on the disease, and the patient on “how to live with it”. However this different view may create an initial gap which needs to be bridged to enhance the therapeutic relationship.Acknowledging the existence of this gap is likely a first step towards an improved patient-doctor communication. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests None Declared.

Background Access to quality disease information is one of the cornerstones of therapeutic education and critical to help the doctor-patient dialog and adherence to treatment. Lupus patients and doctors alike lack the time in consultation to address the many questions patients have. As a result, patients often rely on poor quality or unverified information from the web. For non-English speakers, the language barrier is adding yet another obstacle to quality therapeutic education. Objectives To provide access to doctor verified SLE quality information on-line, in the mother language of 95%+ of the European population so that doctors can refer patients to quality information sources. Methods With the endorsement of ERN ReCONNET reference network, Lupus Europe and doctors from ReCONNET’s SLE Working group have developed a multilingual Euro-wide website answering the top 100 questions patients have about lupus. The starting point to build the content has been the French book “lupus en 100 questions” authored by lupus experts from the French FAI2R. Working extensively with patients from Lupus Europe PAN, the list of questions has been adjusted, answers to existing questions have been checked and validated or adjusted for up-to-dateness. New patient questions have been answered by lupus specialised doctors in collaboration with patients. The resulting documents have been translated in English and the translations validated by both patients and doctors. This core English version has then received feedback from ERN ReCONNET SLE WG doctors. Finally, the end result has been translated in more than 15 languages, verified by native lupus doctors and patients prior to being put on line. This exercise is continuing at the time of writing the abstract, with the goal of covering more than 20 languages and 99% of European population by end 2023. The web site is managed and controlled by Lupus Europe, the European Lupus patients organisation. Aside from this “one-shot” effort, a process has been designed to collect questions and comments from patients and doctors. Those will then feed the on-going maintenance of the information. New developments or new questions will be used to systematically review the content and update it using a Delphi process within the ERN ReCONNET SLE Working group. Results lupus100.org website provides free of charge quality information on lupus for patients, relatives, doctors or students. Its multilingual character allows all people in Europe to have access to verified information in their own language, with lay terms. This non-commercial tool is available for doctors of all countries to grow their patients education on lupus and hence build a stronger therapeutic alliance, minimising the time lost in fighting wrong information. Conclusion A new tool is available for doctors and patients to build lupus education. The challenges are now (a) to maximise its reach by ensuring all patients and doctors are aware of the initiative, and (b) to ensure that currency is maintained medium to long term. Reference [1]www.lupus100.org Acknowledgements: NIL. Disclosure of Interests None Declared.

ABSTRACTObjectiveDespite significant improvements in diagnosis delay and treatment strategies, the burden of Systemic Lupus Erythematosus (SLE) remains high. The objective of the study was to assess the association between diagnosis delay, disease activity and burden on daily life (BoDL) in a large sample of European patients with SLE.MethodsIn May 2020, Lupus Europe, the European umbrella patient association for SLE, conducted a multilingual anonymous online cross-sectional study to individuals with a self-reported physician’s diagnosis of SLE living in Europe. The BoDL score was computed using 1 to 5 Likert scales on 5 domains (mobility, anxiety/depression, self-care, daily activities and pain/discomfort) and the sum was rescaled on a 0 (minimum Burden on daily life) to 100 (maximum BoDL) scale. Comparisons between independent groups were made using the Mann-Whitney test for continuous outcomes and the Chi-2 test (or Fisher’s exact test) for quantitative data.ResultsData of 4,150 SLE patients from 35 European countries were analysed. Those with a diagnosis of SLE within 2 years of first symptoms had significantly lower mean BoDL scores than those diagnosed after 5 years (33.6 versus 44.0, p<0.001). The BoDL score was better in SLE patients feeling that their lupus had been under control during the last 3 months versus the others (34.0% versus 47.6%, p<0.001).ConclusionThis large international study highlights the association between diagnosis delay and self-perceived disease activity with the burden of the disease on the daily life of people living with SLE. Healthcare pathways, which may accelerate diagnosis and optimize therapeutic management, are necessary to improve patients’ outcomes in SLE.

The Control In Steel project, a cooperation of four research institutes, revisited research projects of the last 20 years focusing on automation and control solutions applied to the downstream steel production route. During this investigation we found hints to those solutions, which were beneficial for specific problems. For our analysis, 46 projects were systematically reviewed. Taxonomies for the problem space, the solution space, the barriers and issues and the impact were developed and each project categorized along these taxonometrical dimensions. As a result, the interdependencies between solutions and impact could be analysed in a quantifiable way, which led to a new way of evaluating project success. It also brought new insights about the most promising techniques already applied and those techniques, that have been apparently not yet been applied to steel production, although being highly successful in other domains. This leads to potential future research chances for the steel production and their complex process chains. The paper will also finally demonstrate how a similar taxonometrical approach can be used to conserve expert knowledge in automation to feed a truly artificially intelligent control solution – not only exploiting machine learning methods but essentially using machine reasoning on top of the digitized expert knowledge to achieve improved process automation.

Resource consumption is an important topic for steelmaking industry, which is spending significant efforts to reduce its environmental impact and improve its competitiveness. Water is largely exploited in steelworks for indirect and direct cooling, specific surface treatment, and fumes washing and cooling. It is already reused and recycled after restoring its quality through treatments for temperature and/or pollutant reduction. However, sometimes water networks are not optimized due to outdated water treatments, lack of continuous monitoring, and water network management strategies often based on experience without automation. In recent years, new water treatments, simulation, and optimization tools are becoming available, together with a stronger awareness of the importance of online parameters monitoring. Therefore, improvement of water cleaning, reuse, recycling, and consequent reduction of impact related to water exploitation are potentially achievable. The introduction of innovative treatments must be tested before their implementation in steel plants and the exploration of their behavior in different operating conditions is fundamental. The presented work addresses this topic through the application of several models of operational units, developed in OpenModelica environment and aggregated into a plant simulator. The simulator was used in different case studies related to an Italian plant to assess the impact of new filtering technology for reducing suspended solids on the analyzed water networks and test the effects of different operating configurations on the treatment efficiency. The introduction of new filtration technology leads to environmental and economic advantages due to freshwater intake reduction and water management improvement.Copyright (c) 2022 The Authors. This is an open access article under the CC BY-NC-ND license (https://creativecommons.org/licenses/by-nc-nd/4.0/)

OBJECTIVE:To develop recommendations for cardiovascular risk (CVR) management in gout, vasculitis, systemic sclerosis (SSc), myositis, mixed connective tissue disease (MCTD), Sjögren's syndrome (SS), systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). METHODS:Following European League against Rheumatism (EULAR) standardised procedures, a multidisciplinary task force formulated recommendations for CVR prediction and management based on systematic literature reviews and expert opinion. RESULTS:Four overarching principles emphasising the need of regular screening and management of modifiable CVR factors and patient education were endorsed. Nineteen recommendations (eleven for gout, vasculitis, SSc, MCTD, myositis, SS; eight for SLE, APS) were developed covering three topics: (1) CVR prediction tools; (2) interventions on traditional CVR factors and (3) interventions on disease-related CVR factors. Several statements relied on expert opinion because high-quality evidence was lacking. Use of generic CVR prediction tools is recommended due to lack of validated rheumatic diseases-specific tools. Diuretics should be avoided in gout and beta-blockers in SSc, and a blood pressure target <130/80 mm Hg should be considered in SLE. Lipid management should follow general population guidelines, and antiplatelet use in SLE, APS and large-vessel vasculitis should follow prior EULAR recommendations. A serum uric acid level <0.36 mmol/L (<6 mg/dL) in gout, and disease activity control and glucocorticoid dose minimisation in SLE and vasculitis, are recommended. Hydroxychloroquine is recommended in SLE because it may also reduce CVR, while no particular immunosuppressive treatment in SLE or urate-lowering therapy in gout has been associated with CVR lowering. CONCLUSION:These recommendations can guide clinical practice and future research for improving CVR management in rheumatic and musculoskeletal diseases.

A clinical guideline is a document with the aim of guiding decisions based on evidence regarding diagnosis, management and treatment in specific areas of healthcare. Specific to rheumatic and musculoskeletal diseases (RMDs), adherence to clinical guidelines recommendations impacts the outcomes of people with these diseases. However, currently, the implementation of recommendations is less than optimal in rheumatology.The WHO has described the implementation of evidence-based recommendations as one of the greatest challenges facing the global health community and has identified the importance of scaling up these recommendations. But closing the evidence-to-practice gap is often complex, time-consuming and difficult. In this context, the implementation science offers a framework to overcome this scenario.This article describes the principles of implementation science to facilitate and optimise the implementation of clinical recommendations in RMDs. Embedding implementation science methods and techniques into recommendation development and daily practice can help maximise the likelihood that implementation is successful in improving the quality of healthcare and healthcare services.

Objective To update the 2012 EULAR/ERA–EDTA recommendations for the management of lupus nephritis (LN). Methods Following the EULAR standardised operating procedures, a systematic literature review was performed. Members of a multidisciplinary Task Force voted independently on their level of agreeement with the formed statements. Results The changes include recommendations for treatment targets, use of glucocorticoids and calcineurin inhibitors (CNIs) and management of end-stage kidney disease (ESKD). The target of therapy is complete response (proteinuria <0.5–0.7 g/24 hours with (near-)normal glomerular filtration rate) by 12 months, but this can be extended in patients with baseline nephrotic-range proteinuria. Hydroxychloroquine is recommended with regular ophthalmological monitoring. In active proliferative LN, initial (induction) treatment with mycophenolate mofetil (MMF 2–3 g/day or mycophenolic acid (MPA) at equivalent dose) or low-dose intravenous cyclophosphamide (CY; 500 mg × 6 biweekly doses), both combined with glucocorticoids (pulses of intravenous methylprednisolone, then oral prednisone 0.3–0.5 mg/kg/day) is recommended. MMF/CNI (especially tacrolimus) combination and high-dose CY are alternatives, for patients with nephrotic-range proteinuria and adverse prognostic factors. Subsequent long-term maintenance treatment with MMF or azathioprine should follow, with no or low-dose (<7.5 mg/day) glucocorticoids. The choice of agent depends on the initial regimen and plans for pregnancy. In non-responding disease, switch of induction regimens or rituximab are recommended. In pure membranous LN with nephrotic-range proteinuria or proteinuria >1 g/24 hours despite renin–angiotensin–aldosterone blockade, MMF in combination with glucocorticoids is preferred. Assessment for kidney and extra-renal disease activity, and management of comorbidities is lifelong with repeat kidney biopsy in cases of incomplete response or nephritic flares. In ESKD, transplantation is the preferred kidney replacement option with immunosuppression guided by transplant protocols and/or extra-renal manifestations. Treatment of LN in children follows the same principles as adult disease. Conclusions We have updated the EULAR recommendations for the management of LN to facilitate homogenization of patient care.

Background:Up to 40% of systemic lupus erythematosus (SLE) patients develop kidney disease, which represents a major cause of morbidity.Objectives:To update the 2012 EULAR/ERA-EDTA recommendations for the management of lupus nephritis (LN).Methods:We followed the EULAR standardised operating procedures for the publication of treatment recommendations. Delphi-based methodology led to 15 questions for systematic literature review (SLR), which was undertaken by three fellows.Results:The changes include recommendations for treatment targets, use of glucocorticoids and calcineurin inhibitors (CNI), and management of end-stage-kidney-disease (ESKD). The target of therapy is complete response (proteinuria <0.5-0.7gr/24h with [near-]normal glomerular filtration rate) by 12 months, but this can be extended in patients with baseline nephrotic-range proteinuria. Hydroxychloroquine is recommended with regular ophthalmological monitoring. In active proliferative LN, initial (induction) treatment with mycophenolate mofetil (MMF 2-3g/day, or mycophenolic acid at equivalent dose) or low-dose intravenous cyclophosphamide (CY; 500mg x6 biweekly doses), both combined with glucocorticoids (pulses of intravenous methylprednisolone, then oral prednisone 0.3-0.5mg/kg/day) is recommended. MMF/CNI (especially tacrolimus) combination and high-dose CY are alternatives, for patients with nephrotic-range proteinuria and adverse prognostic factors. Subsequent long-term maintenance treatment with MMF or azathioprine should follow, with no or low-dose (<7.5 mg/day) glucocorticoids. The choice of agent depends on the initial regimen and plans for pregnancy. In non-responding disease, switch of induction regimens or rituximab are recommended. In pure membranous LN with nephrotic-range proteinuria or proteinuria >1g/24h despite renin-angiotensin-aldosterone blockade, MMF in combination with glucocorticoids is preferred. Assessment for kidney and extra-renal disease activity, and management of comorbidities is lifelong with repeat kidney biopsy in cases of incomplete response or nephritic flares. In ESKD, transplantation is the preferred kidney replacement option with immunosuppression guided by transplant protocols and/or extra-renal manifestations.Conclusion:The updated recommendations intend to inform rheumatologists, nephrologists, patients, national professional societies, hospital officials, social security agencies and regulators about the treatment of LN based on most recent evidence.Disclosure of Interests:Antonis Fanouriakis Paid instructor for: Paid instructor for Enorasis, Amgen, Speakers bureau: Paid speaker for Roche, Genesis Pharma, Mylan, Myrto Kostopoulou: None declared, Kim Cheema: None declared, Hans-Joachim Anders: None declared, Martin Aringer Consultant of: Boehringer Ingelheim, Roche, Speakers bureau: Boehringer Ingelheim, Roche, Ingeborg Bajema Consultant of: GSK, John N. Boletis Grant/research support from: GSK, Pfizer, Paid instructor for: GSK, Abbvie, UCB, Enorasis, Eleni Frangou: None declared, Frederic Houssiau Grant/research support from: UCB, Consultant of: GSK, Jane Hollis: None declared, Alexandre Karras: None declared, Francesca Marchiori: None declared, Stephen Marks: None declared, Gabriela Moroni: None declared, Marta Mosca: None declared, Ioannis Parodis: None declared, Manuel Praga: None declared, Matthias Schneider Grant/research support from: GSK, UCB, Abbvie, Consultant of: Abbvie, Alexion, Astra Zeneca, BMS, Boehringer Ingelheim, Gilead, Lilly, Sanofi, UCB, Speakers bureau: Abbvie, Astra Zeneca, BMS, Chugai, GSK, Lilly, Pfizer, Sanofi, Josef S. Smolen Grant/research support from: AbbVie, AstraZeneca, Celgene, Celltrion, Chugai, Eli Lilly, Gilead, ILTOO, Janssen, Novartis-Sandoz, Pfizer Inc, Samsung, Sanofi, Consultant of: AbbVie, AstraZeneca, Celgene, Celltrion, Chugai, Eli Lilly, Gilead, ILTOO, Janssen, Novartis-Sandoz, Pfizer Inc, Samsung, Sanofi, Vladimir Tesar: None declared, Maria Trachana: None declared, Ronald van Vollenhoven Grant/research support from: AbbVie, Amgen, Arthrogen, Bristol-Myers Squibb, GlaxoSmithKline (GSK), Janssen Research & Development, LLC, Lilly, Pfizer, Roche, and UCB, Consultant of: AbbVie, AstraZeneca, Biotest, Bristol-Myers Squibb, Celgene, Crescendo Bioscience, GSK, Janssen, Lilly, Medac, Merck, Novartis, Pfizer, Roche, UCB and Vertex, Speakers bureau: AbbVie, AstraZeneca, Biotest, Bristol-Myers Squibb, Celgene, Crescendo Bioscience, GlaxoSmithKline, Janssen, Lilly, Merck, Novartis, Pfizer, Roche, UCB, Vertex, Alexandre Voskuyl: None declared, Y.K. Onno Teng Grant/research support from: GSK, Consultant of: GSK, Aurinia Pharmaceuticals, Novartis, Bernadette van Leeuw: None declared, George Bertsias Grant/research support from: GSK, Consultant of: Novartis, David Jayne Grant/research support from: ChemoCentryx, GSK, Roche/Genentech, Sanofi-Genzyme, Consultant of: Astra-Zeneca, ChemoCentryx, GSK, InflaRx, Takeda, Insmed, Chugai, Boehringer-Ingelheim, Dimitrios Boumpas: None declared

The objective was to develop evidence-based recommendations for the management of antiphospholipid syndrome (APS) in adults. Based on evidence from a systematic literature review and expert opinion, overarching principles and recommendations were formulated and voted. High-risk antiphospholipid antibody (aPL) profile is associated with greater risk for thrombotic and obstetric APS. Risk modification includes screening for and management of cardiovascular and venous thrombosis risk factors, patient education about treatment adherence, and lifestyle counselling. Low-dose aspirin (LDA) is recommended for asymptomatic aPL carriers, patients with systemic lupus erythematosus without prior thrombotic or obstetric APS, and non-pregnant women with a history of obstetric APS only, all with high-risk aPL profiles. Patients with APS and first unprovoked venous thrombosis should receive long-term treatment with vitamin K antagonists (VKA) with a target international normalised ratio (INR) of 2–3. In patients with APS with first arterial thrombosis, treatment with VKA with INR 2–3 or INR 3–4 is recommended, considering the individual’s bleeding/thrombosis risk. Rivaroxaban should not be used in patients with APS with triple aPL positivity. For patients with recurrent arterial or venous thrombosis despite adequate treatment, addition of LDA, increase of INR target to 3–4 or switch to low molecular weight heparin may be considered. In women with prior obstetric APS, combination treatment with LDA and prophylactic dosage heparin during pregnancy is recommended. In patients with recurrent pregnancy complications, increase of heparin to therapeutic dose, addition of hydroxychloroquine or addition of low-dose prednisolone in the first trimester may be considered. These recommendations aim to guide treatment in adults with APS. High-quality evidence is limited, indicating a need for more research.