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Pregnancy is one of the most challenging processes the human body is exposed to: the healthy mother can carry to term a genetically different new-born, while her immune system adapts to tolerate this new status and avoids rejection. In autoimmune disorders, motherhood is even more challenging, with additional medical counselling, mother care, and foetus development checks being necessary. While the aspects of supplementary mother care and pregnancy progress tracking are associated with well-established medical procedures and protocols, counselling, be it pre- or post-conception, is still underestimated and scarcely applied. Indeed, over the past decades, medical counselling for this particular population has changed significantly, but from a healthcare’s provider point of view, more is required to ensure a smooth, controllable pregnancy evolution. One of the most frequent autoimmune diseases affecting young females during their fertile years is Systemic Lupus Erythematosus (SLE). Like other heterogenous diseases, it exposes the mother to severe, organ-threatening complications and unpredictable evolution. Both the disease and its treatment can significantly affect the mother’s willingness to engage in a potentially risky pregnancy, as well as the likeliness to carry it to term without any impairments. A good collaboration between the patient’s rheumatologist and obstetrician is therefore mandatory in order to: (a) allow the mother to make an informed decision on pursuing with the pregnancy; (b) ensure a perfect synchronization between pregnancy terms and treatment; and (c) avoid or minimize potential complications. The best approach to achieve these outcomes is pregnancy planning. Moreover, knowing one desired prerequisite for a successful pregnancy evolution in SLE mothers is a stable, inactive, quiescent disease for at least six months prior to conception, planning becomes more than a recommended procedure. One particular aspect that requires attention before conception is the treatment scheme applied before delivery as autoantibodies can influence significantly the course of pregnancy. In this view, future SLE mothers should ideally benefit from preconception counselling within their agreed care pathway. A multidisciplinary team including at least the rheumatologist and obstetrician should be employed throughout the pregnancy, to decide on the appropriate timing of conception and compatible medication with respect to disease activity, as well as to monitor organ involvement and foetus development progress.

Background: In primary Sjögren’s Syndrome (pSS), cutaneous vasculitis lesions (CVL) are extraglandular manifestations with an important clinical and prognostic impact and their early detection might contribute to the improvement of disease control and even patients’ survival. The aim of this study was to evaluate the predictive potential of hematological elements in the development of CVL in pSS patients. Methods: In this single center, retrospective study, a total of 245 participants were included (124 pSS patients and 121 healthy controls). Complete blood count, inflammatory and immunological parameters were determined at the initial visit. pSS patients underwent a periodical follow-up program, when disease progression and response to therapy was monitored, including the emergence of CVL. Results: In pSS, leucocytes, lymphocyte, neutrophil, monocyte, erythrocyte and platelet counts are significantly decreased compared to healthy subjects (p < 0.001), whereas cellular ratios: NLR, PLR, MLR, and immunological and inflammatory parameters are significantly increased (p < 0.001). A total of 34 patients with pSS (27.41%) developed CVL during the follow-up period. The occurrence of CVL was positively correlated with neutrophil and platelet counts (p < 0.001), while for lymphocytes the correlation was negative (p < 0.001). Cellular ratios: NLR, PLR and MLR, and gammaglobulins also revealed significant positive correlations with the emergence of CVL in pSS (p < 0.001). The multivariate analysis confirmed the independent predictive character for CVL emergence in pSS for NLR (CI95% 0.053–0.2, p < 0.002), PLR (CI95% 0.001–0.003, p < 0.003), MLR (CI95% 0.086–0.935, p < 0.019), and gammaglobulins (CI95% 0.423–0.688, p < 0.001). Conclusions: Standard hematological parameters, widely used in the assessment of pSS patients, such as NLR, PLR, MLR and gammaglobulins could become valid elements that might be used for the early detection of patients at risk for the development of CVL.

Objective. To evaluate sexual function in a cohort of men with rheumatic disease compared to healthy controls. Patients and methods. The study included 60 patients (ankylosing spondylitis, rheumatoid arthritis, systemic sclerosis, gout, psoriatic arthritis) and 60 healthy age-matched controls. Sexual function of patients and controls was evaluated by the Sexual Health Inventory for Men and a detailed questionnaire. Results. The mean age of study group was 45,26 (7.8) years. In 90% of the cases sexual life changed after diagnosis: only 1.67% described their sexual life as being very satisfying, while 21.67% as being satisfying, 68.33% as not satisfying and 8.33% as absent. 40% of the patients had diminished libido, significantly lower compared to the study group (p = 0.043). Most patients presented with mild erectile dysfunction (60%), while most of the systemic sclerosis patients presented with mild to moderate erectile dysfunction. Erectile dysfunction scores were significantly lower compared to controls (0.009). 55% of the patients reported no influence of the treatment on their sexual life, 38% of the patients reported that specific treatment improved their sexual life.There was no statistical difference between SHIM scores of patients using biological therapies and other drugs. Conclusions. This work suggests that men with rheumatic conditions have a worse sexual function in comparison with healthy control. However, specific disease parameters or treatments were not linked to sexual dysfunction.

Availability of biosimilar medicines is rapidly increasing, potentially leading to a wider prescription and a support for healthcare system costs. Despite efforts of patient and physician educational initiatives there still remains a reluctance in prescribing or administering this type of products. A multi-centric survey was conducted on 336 patients with rheumatoid arthritis (RA), spondyloarthritis (SpA), psoriatic arthritis (PsA) or lupus (SLE) on biologic treatment. Half of the patients stated never hearing of biosimilars and one third feared adverse events (infections, cancer). Another 20% felt insecure on drug tolerability or efficacy. However, the majority declared relying on their rheumatologist if prescribing a biosimilar. Lack of patient information should be accurately addressed by health organizations so that the choice of treatment is done knowingly.

ObjectiveThe present paper describes the design, development, and implementation of the AutoInflammatory Disease Alliance (AIDA) International Registry specifically dedicated to patients with Schnitzler's syndrome.MethodsThis is a clinical physician-driven, population- and electronic-based registry implemented for the retrospective and prospective collection of real-life data from patients with Schnitzler's syndrome; the registry is based on the Research Electronic Data Capture (REDCap) tool, which is designed to collect standardized information for clinical research, and has been realized to change over time according to future scientific acquisitions and potentially communicate with other existing or future similar registries.ResultsSince its launch, 113 centers from 23 countries in 4 continents have been involved. Fifty-seven have already obtained the approval from their local Ethics Committees. The platform counts 324 users (114 Principal Investigators, 205 Site Investigators, 2 Lead Investigators, and 3 data managers) at current (April 28th, 2022). The registry collects baseline and follow-up data using 3,924 fields organized into 25 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, laboratory, instrumental exams, therapies, socioeconomic information, and healthcare access.ConclusionsThis International Registry for patients with Schnitzler's syndrome facilitates standardized data collection, enabling international collaborative projects through data sharing and dissemination of knowledge; in turn, it will shed light into many blind spots characterizing this complex autoinflammatory disorder.

Although perceived as an adaptative immune disorder, mainly related to Lymphocyte B and T, last years focus on Systemic Lupus Erythematosus (SLE) pathogeny emphasised the important role of innate immunity. This should not take us by surprise since the lupus cell described by Hargraves and colleagues in 1948 was a neutrophil or macrophage with specific aspect after coloration with haematoxylin related to cell detritus engulfment (Hargraves et al., 1948) [1] (

Behçet’s disease is a systemic vasculitis with several well-defined organ manifestations, including various mucocutaneous features. Among them, the urinary tract involvement is rarely cited, most data focusing on bladder dysfunction due to neuroBehçet. This article presents a rare case of a young female patient with urological complaints that was diagnosed with right ureteral ulceration, later confirmed as vasculitis at the histopathological examination. Urological intervention together with adequate immunosuppression let to the healing of the ulcerative lesion. The unusual vasculitic lesion site indicates the complexity of Behçet’s disease that requires careful investigation and treatment.

Background: No fully validated index is available for assessing overall disease activity in systemic sclerosis (SSc). Objectives: To estimate the effect of disease activity as measured by different disease activity indices on the risk of subsequent organ damage. Methods: The European Systemic sclerosis study group activity index (EScSG AI), the European Scleroderma Trials and Research Group Activity Index (r-EUSTAR AI), 12 point activity index proposed by Minier (12point AI) were calculated for 91 patients; the CRISS (The Composite Response Index for Systemic Sclerosis) for patients included after 2016. Data were analysed by parametric and non-parametric tests and logistic regression. Results: EscSG AI, r-EUSTAR AI and 12point AI correlated with lung involvement. EScSG AI and r-EUSTAR AI correlated with diffuse skin involvement. EscSG AI correlated with digital ulcers and diffuse cutaneous involvement and r-EUSTAR AI with a renal crisis. Bivariate analysis showed an inverse correlation between the three disease activity scores and forced vital capacity (FVC) (p Conclusion: Our study could not establish a gold standard to assess disease activity in SSc; especially EscSG AI and r-EUSTAR AI could quantify and predict major organ involvement in daily practice. CRISS can be useful as an outcome measure for patients with short disease duration included in clinical studies.

The usefulness of capillaroscopy in the follow-up of scleroderma patients and the possible prognostic role for the appearance of visceral involvement is suggested by many authors but still under debate.The aim of this study was to assess the role of monitoring capillaroscopic abnormalities (qualitative and semiquantitative) in relation with parameters of interstitial lung involvement and pulmonary arterial hypertension(PAH). A strong correlation was identified between initial capillaroscopy scores and FVC (r=-.47, p=0.002), DLCO (r=- .51, p< 0.001) and sPAP (r=0.34, p1) was asociated with worsening of FVC (r=0.32,p

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by the production of autoantibodies, which deposit within tissues and fix complement leading to systemic inflammation (1). Is a heterogeneous disease with a continuum of disease activity. Some patients can have predominant skin and joint involvement, whereas others can present with organ-threatening diseases such as nephritis, cardiac involvement or even neurologic manifestations. Relatives of patients with SLE appear to be at higher risk of SLE and other autoimmune diseases, but estimates of individual familial risks are largely unavailable or unreliable (2,3). The purpose of ANA (antinuclear antibody) determination is generally to screen patients suspected from generalised autoimmune diseases, that is, systemic lupus erythematosus (SLE), rheumatoid arthritis, Sjögren’ s syndrome, scleroderma, polymyositis, or mixed connective tissue disease. Clinical and paraclinical studies are needed to reach a definitive diagnosis.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by the production of autoantibodies, which deposit within tissues and fix complement leading to systemic inflammation (1). Is a heterogeneous disease with a continuum of disease activity. Some patients can have predominant skin and joint involvement, whereas others can present with organ-threatening diseases such as nephritis, cardiac involvement or even neurologic manifestations. Relatives of patients with SLE appear to be at higher risk of SLE and other autoimmune diseases, but estimates of individual familial risks are largely unavailable or unreliable (2,3).

Autoimmune rheumatic disorders have a multifactorial determinism, caused by various environmental factors acting on the individual’s genetic susceptibility, destabilizing the systems which regulate the immune response. Epidemiological and genetic investigations are very important to demonstrate the contribution of genetic factors to the development of these autoimmune diseases. The contribution of genetic factors in causing autoimmune diseases has been demonstrated by familial aggregation. Moreover, it was also quantified by determining heritability, expressing the proportion of genetic factors in the etiology. It is now clear that common genes underlie multiple autoimmune disorders.

Introduction. Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) represent a group of conditions evolving with necrotizing inflammation in small and medium-sized blood vessels. AAV are composed of GPA (granulomatosis with polyangiitis, former Wegener’s granulomatosis), MPA (microscopic polyangiitis) and EGPA (eosinophilic granulomatosis with polyangiitis, former Churg-Strauss syndrome). AAV receive immunosuppressive therapy associated with a high risk of complications. Objective. The aim of this study was to characterize a single center cohort of AAV patients regarding clinical, biological and therapeutic features. Method. We realized a cross-sectional study by consequently enrolling all the patients registered with AAV diagnosis between 2009 and 2017 in Department of Rheumatology of “Sfânta Maria” Hospital. Demographic, diseaserelated and therapeutic-related parameters were collected. The data was extracted from the clinical files. Results. The study sample included 26 cases, 15 females and 11 males: 20 patients GPA, 4 MPA and 2 cases EGPA. Mean age at the time of diagnosis was around 48 but 12 patients presented delays between age at the onset and age at the time of diagnosis (the mean delay was 2 years). The most frequent clinical manifestation identified where pulmonary, musculoskeletal and renal. 15 patients had a diagnostic biopsy performed. ANCA detection revealed 16 cases of c-ANCA and 7 cases of p-ANCA and 11 patients presented other positive serology. A combination of glucocorticoids and cyclophosphamide was used in most of the cases for remission-induction treatment and the same scheme was used for relapse cases. For maintenance phase a combination of glucocorticoids and azathioprine was preferred. 13 patients (50%) developed treatment related complications. Conclusion. Most of the patients were diagnosed with GPA (20) and the least were diagnosed with EGPA (2). Biopsy was performed in 15 cases and it was mostly nasal. For remission-induction prevailed the combination of glucocorticoids and cyclophosphamide. Most treatment related complications were due to glucocorticoids administration. Osteoporosis was predominant.

Background. Behçet’s disease is a rare type of vasculitis. Validated activity and damage scores were developed for vasculitis patients in order to allow a better way to evaluate disease activity and decide treatment plans. Objective. The main objective was to compare two vasculitis activity scores applied to a group of patients diagnosed with Behçet’s disease and establish correlations between them, damage and the need for immunosuppressive therapy. The secondary objective was to evaluate the connection between damage progression, classical immunosuppressant therapy and long-term cortisone use. Methods. A study was performed on a cohort of patients diagnosed with Behçet’s disease according The International Criteria for Behçet‘s Disease (ICBD) under surveillance in one Rheumatology Centre, from a non-endemic area. Vasculitis activity and damage scores were calculated for each patient. Results. 20 patients were included in the study, with a mean age of 35.7 years ± 10.5 years standard deviation(SD), 14 (70%) under the age of 40, with a male predominance 60% (12 patients). All patients presented active disease at the time of the diagnosis. Spearman’s rank correlation coefficient between BVAS v3 and BDCAF was strong r = 0.862 and statistically significant p < 0.001. The outcome analysis after remission was calculated and rank correlation coefficient between VDI, and both BVASv3 and BDCAF was moderate (VDI-BVASv3 r = 0.747, p < 0.001, VDI - BDCAF r = 0.795, p < 0.001). As for immunosuppression induction decision and activity scores, the correlation coefficient was moderate (r = 0.734 for BVASv3, r = 0.647 for BDCAF) with p < 0.001. There was a moderate correlation between immunosuppressive treatment and VDI (r = 0.700, p < 0.001). The cause of damage (i.e. vasculitis vs. treatment) is not taken into consideration when we calculate VDI. Data analysis showed the presence of mild correlation and no statistical impact between cyclophosphamide treatment duration and damage calculated as VDI (r = 0.474, p = 0.36). In contrast, when rank correlation coefficient between cortisone therapy and VDI was calculated, a moderate statistical impact was observed (r = 0.609, p < 0.001). Conclusions. An objective assessment of disease’s activity can be obtained using disease activity indexes. A moderate to strong correlation was obtained between activity indexes, immunosuppressive treatment initiation and damage progression. Comparing the two activity indexes, it resulted that: BVASv3 correlates stronger with the need for immunosuppressive treatment and both of them are equally able to anticipate damage. Damage progression was correlated stronger with long-term cortisone use, rather than immunosuppressive therapy.

Skin involvement in SSc is an important marker of disease activity, severity and prognosis, making the assessment of skin a key issue in SSc clinical research. We reviewed the published data assessing skin involvement in clinical trials and summarized the major conclusions important in SSc clinical research. A systematic literature review identified randomized controlled trials using skin outcomes in SSc. Analysis examined the validity of the different skin measures based on literature findings. Twenty-two randomized controlled trials were found. The average study duration was 10.2 (s.d. 4.5) months, mean (s.d.) sample size 32.4 (32.6) and 26.7 (27.8) in intervention and control arms, respectively. The 17-site modified Rodnan skin score is a fully validated primary outcome measure in diffuse cutaneous SSc. Skin histology seems to be an appropriate method for evaluation of skin thickness. These findings have important implications for clinical trial design targeting skin involvement in SSc.

Familial Mediterranean Fever (FMF) is the most common condition in the group of autoinflammatory diseases. It has a relatively heterogeneous clinical display included in the Tel-Hashomer diagnostic criteria that can be further confirmed by genetic testing showing a mutation in the MEFV gene. AA Amyloidosis is the most sever complication in these patients. Colchicine is the standard treatment and/or alternative biological agents if necessary. This article aims to describe the evolution of a patient with FMF, firstly diagnosed as reactive arthritis, his clinical manifestations and therapeutic options.

Background Currently there is no fully validated index for assessing overall disease activity in patients with systemic sclerosis (SSc). Objectives To estimate the effect of disease activity as measured by 4 disease activity indices on the risk of subsequent organ damage in a EUSTAR center cohort. Methods Longitudinal observational study; European Systemic sclerosis study group disease activity index (EScSG DAI), revised EUSTAR disease activity index (r-EUSTAR DAI), 12 point activity index proposed by Minier (12point DAI) were calculated for all patients; the CRISS (The Combined Response Index for Systemic Sclerosis) only for patients included after 2016. Student t-test/Mann-Whitney test, chi-square test were used to evaluate differences across subgroups; Pearson’s bivariate correlation/Spearman’s rank correlation coefficient to evaluate the association between variables. The predictive value of various variables for major organ involvement was assessed by Roc curves and univariate regression. Results 91 patients were selected,77 females (84,61%), 51,65(13,20) years old at diagnosis, 49,45% diffuse subset. Disease activity scores were all higher in male patients and in patients with diffuse cutaneous involvement, digital ulcers(DU), lung fibrosis, scleroderma renal crisis (SRC), arrythmias, muscle atrophy, gastric involvement, antitopoisomerase-1 positive, EscSG DAI correlated with forced vital capacity (FVC)(r=0.73,p R- EUSTAR DAI correlated with FVC(r=0.6,p 12 point DAI correlated with FVC(r=0.57,p EscSG predicted well lung fibrosis (AUC=0.79,p 12point DAI was a good predictor for lung fibrosis(AUC=0.74,p In the regression analysis, lung fibrosis(beta=0.5,95%CI=1,21-2,58,p The CRISS cohort included 35 patients, 32 females(91,42%), 48.48(14.24) years old, 62.85% diffuse subset, medium disease duration 11.88(7.9) months. 8 patients were excluded due to new onset or worsening of lung fibrosis or SRC. None of the patients had a CRISS score with a probability of improvement>0.6. Conclusion We could not conclude that there is a gold standard to measure disease activity; for daily practice especially EscSG and r-EUSTAR DAI quantify and predict major organ involvement. CRISS can be useful as an outcome measure for patients with short disease duration and closely monitored in clinical studies. References [1] K. Melsens, et al. Disease activity indices in systemic sclerosis: a systematic literature review Clin Exp Rheumatol2016; 34 (Suppl. 100):S186-S192. Disclosure of Interests Laura Groseanu: None declared, Sorana Petrescu: None declared, Andra Balanescu Speakers bureau: multiple, Daniela Opris-Belinski Grant/research support from: GLORIA, Speakers bureau: multiple, Violeta Bojinca Speakers bureau: multiple, Florian Berghea: None declared, Ioana Saulescu: None declared, Sanziana Daia-Iliescu: None declared, Diana Mazilu Speakers bureau: Pfizer, UCB, Andreea Borangiu: None declared, Cosmin Constantinescu: None declared, Maria Magdalena Negru: None declared, Mihai Abobului: None declared, Violeta Vlad: None declared, Ruxandra Ionescu: None declared

Background: The usefulness of capillaroscopy in the follow-up of scleroderma (SSc) patients and the possible prognostic role for the appearance of visceral involvement is suggested by many authors but still under debate. Objectives: The aim of this study was to assess the role of monitoring capillaroscopic abnormalities (qualitative and semiquantitative) in relation with parameters of interstitial lung involvement and pulmonary arterial hypertension(PAH). Methods: Longitudinal prospective study, 118 SSc patients, follow-up 2000-2017 based on MEDS evaluation sheets. Student t-test/Mann-Whitney test, chi-square test were used to evaluate differences across subgroups. Pearson’s bivariate correlation/Spearman’s rank correlation coefficient were used to evaluate the association between variables. Nailfold videocapillaroscopy (NVC) was performed by a trained physician: qualitative (scleroderma pattern) and semiquantitative scoring (microangiopathy evolution score-MES) were evaluated. Results: 118 patients were included, 103 females (87,29%), 50,58(12,71) years old at diagnosis, 53.39% diffuse subset, mean disease duration 2,81(4.3) years, mean follow-up duration 4.42(3.16)years. At baseline 43.22% of the patients had lung fibrosis,18.64% had PAH, 9,32% had both. At first capillaroscopic evaluation: 12.7% had early, 46.61% active and 37.29% late pattern, medium MES was 4,76(1.43). Baseline active and late capillaroscopic pattern were correlated with lung fibrosis (χ2=9.62, p=0.047) and PAH (χ2=15,36, p=0.004). A strong correlation was identified between initial MES and FVC (r=-.47, p=0.002), FEV(r=-.45, p=0.003), DLCO (r=-.51, p At the 2nd evaluation 5.93% of the patients had early, 0.85% active and 54,24% late scleroderma pattern, mean MES was 5.88(1.74). 19.49% had one step progression of capillaroscopic pattern, 1.69% had 2 steps progression. Lung involvement worsening was defined as decrease of FVC >15%, DLCO>10%, new diagnosis of alveolitis on high resolution computerized tomography or new diagnosis of PAH. Active and late capillaroscopic pattern were correlated with diagnosis of lung fibrosis (χ2=14, p=0.007) and pulmonary hypertension at follow-up examinations (χ2=14,2, p=0.007). Progression of capillaroscopic pattern at follow-up evaluations was not correlated with significant worsening of lung volumes, DLCO, sPAP. Instead, progression of MES (>1) was asociated with worsening of FVC (r=0.32,p Conclusion: Active and late capillaroscopic pattern are associated with increased frequency of interstitial lung disease and pulmonary hypertension in SSc patients. Semiquantitative scoring (microangiopathy evolution score), rather then qualitative capillaroscopic assessment can have a predictive role for new involvement or worsening of previous lung involvement (especially interstitial lung disease) in scleroderma patients, confirming the putative role of capillaroscopy as biomarker in SSc. References [1] Sulli a How to Score the Qualitative Capillaroscopic Patterns in Systemic Sclerosis annals of the Rheumatic Diseases 2013;72:A11 Disclosure of interests: Laura Groseanu: None declared, Patricia Paraschiva: None declared, andra Balanescu Speakers bureau: multiple, Violeta Bojinca Speakers bureau: multiple, Daniela Opris-Belinski Grant/research support from: GLORIA, Speakers bureau: multiple, Florian Berghea: None declared, Diana Mazilu Speakers bureau: Pfizer, UCB, Sanziana Daia-Iliescu: None declared, Ioana Saulescu: None declared, andreea Borangiu: None declared, CLAUDIA COBILINSCHI: None declared, Maria Magdalena Negru: None declared, cosmin-laurentiu constantinescu: None declared, Violeta Vlad: None declared, Mihai abobului: None declared, Ruxandra Ionescu: None declared

Background: Biosimilars are highly similar to original biological medicines1.Worldwide prescription of biosimilars is still low because of the lack of understanding in manufacturing and approval process together with mistrust in the extrapolation of indication in clinical use2.Lack of biosimilar awareness throughout rheumatic patients results in an unjustified underuse2. Objectives: To assess patients’ current knowledge and concerns on biosimilars and to investigate their expectations when receiving such a treatment following the principle of shared-decision making. Methods: A national,cross-sectional survey was conducted in Romanian patients with rheumatoid arthritis(RA),spondyloarthritis(SpA),psoriatic arthritis(PsA)currently on a bio-originator or biosimilar.336 patients responded to a survey distributed from August to December 2018,main topics being patient basic information on biosimilars,their efficacy,safety,price or differences to original drugs. Results: Out of 336 patients,47.3% had RA,39.8% SpA,12.5% PsA with a mean age of 52.5.In the study cohort,13% received approved biosimilars while 87% bio-originators with different mechanisms of action.A yes/no type of question divided patients into those aware or not of biosimilars with further exclusion of those with lack of information.Half of the patients(48.8%) stated they never heard of biosimilars.Surprisingly, four of them were already on this type of treatment.Out of the 172 remaining patients,28.4% feared the risk of adverse events like infections or cancer while almost 20% expressed either insecurity on drug tolerability or the possibility that the biosimilar might be less efficient that the original drug.Another 19.7% certified they had no concerns related to these products and only 15.1% stated confusion regarding the potentially difference in the pharmacological structure of the drugs.Most patients(48.2%) are convinced that the price of a drug should not exceed its efficacy or safety.Half of the respondents say they could accept a switch from an original to a biosimilar if their rheumatologist advises them and 30% might agree but only after being informed.8.7% are interested in scientific proof of the drug and only 1% would consent to a change directly from the pharmacist.When handling prescription,37.7% of patients would want to know if it is an original drug or a biosimilar while 20% do not mind if they receive either.Another 30% trust their rheumatologist and 12.7% would feel more secure if receiving a patient card and written information.Most patients(73.2%)say that they feel completely confident in their rheumatologist if they would want to prescribe a biosimilar,18.6% will have doubts but they will accept the drug and 4% would ask for another medical opinion.After biosimilar initiation,45.9% would be cautious when administrating it,23.2% would stop the drug if an adverse event occured and 15% would have no fears. Conclusion: Study results confirm there is still a significant information gap concerning biosimilars in patient population.Most concerns on biosimilars are related to adverse event occurrence.There is a need to improve patient education on biosimilars involving patients and health professionals.Shared-decision principle is more of a myth since most patients rely entirely on their physician for prescribing the most appropriate product. Reference [1] Declerck P, et al. The Language of Biosimilars:Clarification,Definitions and Regulatory Aspects.Drugs 2017. 2.O’Callaghan J, et al. Regulatory Science Ireland:bridging the information gap on biosimilar medicines.GaBI J 2016. Disclosure of Interests: CLAUDIA COBILINSCHI: None declared, Daniela Opris-Belinski Grant/research support from: GLORIA, Speakers bureau: multiple, Catalin Codreanu: None declared, Razvan Ionescu Speakers bureau: multiple, Claudia Mihailov Speakers bureau: Abbvie, Pfizer, Novartis, MSD, Roche, Biogen, UCB, Lilly, Magda Parvu: None declared, Horatiu Popoviciu: None declared, Elena Rezus: None declared, Ruxandra Ionescu: None declared

Background:The term elderly onset of rheumatoid arthritis (EORA) refers to patients with rheumatoid arthritis (RA) onset after the age of 60. Data published in the literature suggest a special clinical pattern and different prognostic factors in this class of patients.Objectives:To analyze prospectively a cohort of patients diagnosed with EORA, their disease particularities, comorbidities and treatment.Methods:This cohort included consecutive EORA patients, diagnosed and treated in “Sfanta Maria” Clinical Hospital, Bucharest, Romania. The study was conducted for 2 years. Demographic, clinical and laboratory data was obtained. Disease activity was assessed using Disease Activity Score of 28 joints with erythrocyte sedimentation rate (DAS28-ESR). The patients were monitored using disease activity, treatment schedule modifications and possible adverse reactions.Results:The cohort included 110 patients (88 females, 22 males). Their mean age at the beginning of disease manifestations was 70.14 years and the mean age at the diagnosis was 70.85 years. There was no statistical difference regarding the patient’s residential area (urban/rural) and the period between the appearance of clinical signs and the moment of diagnosis confirmation. A great proportion of patients (77 patients, 70%) had seropositive RA, ACPA being found in 84% of the patients with seropositive RA. The mean DAS28-ESR at the diagnosis was 4.44(±1.54). A proportion of 40% of the patients had moderate disease activity, 35 patients (32.73%) - high disease activity, 11 patients (10%) - low disease activity and unexpectedly there were 19 patients (17.27%) in remission at the moment of RA diagnosis. Joint distribution was analyzed: 61.82% patients had large joint involvement, 91.82% - small joint involvement and 53.64 % had mixed joint pattern involvement. A negative significant correlation was found between the small joint involvement pattern and the body mass index (BMI) (p=0.028, R=-0.21). The mean BMI at the diagnosis was 25.81±5.358. Ninety five patients (86,36%) had at least one cardiovascular comorbidity. Hypertension was found in 70% of the patients. Only 4.55% of the patients had rheumatoid nodules and a similar proportion (4.55) had Sjogren syndrome associated. Pulmonary fibrosis was found in only 2 patients. At the moment of diagnosis 50% of patients had anemia, 36.36% had osteoporosis, 25.46% of the patients - hepatic disease, 11.82% - chronic kidney failure and 6.36% were found with a neoplasia. The main conventional synthetic disease modifying drug (csDMARD) that was recommended was methotrexate (81.8%). The second most used csDMARD was hydroxichroloquine (42 patients, 38,18%). The proportion of patients with monotherapy (50%) was similar to that with csDMARD combination (49.09%). During the follow up period only 8 patients (7.27%) had biologic therapy (4 patients - an anti TNF drug). Non steroid anti-inflammatory drugs were used in 46.63%. Cortisone therapy was used for more than 3 months in 80% of the patients. In patients with biologic therapy chronic glucocorticoids were stopped. At least one infection was documented in 20.91% of patients: 2 patients out of 6 patients (33.33%) with biologic DMARD, 14.81% of the patients with csDMARD combination and 21.81% of the patients with csDMARD monotherapy. csDMARD therapy was well tolerated with only 23.63% adverse reactions.Conclusion:Compared to the data published in the literature, in our cohort the rate female:male was higher (4:1). A distinct feature was the high proportion of patients with seropositive RA. The joint pattern seems to be influenced by BMI: small joint pattern is less found in patients with higher BMI. As expected, the patients with EORA had multiple cardiovascular comorbidities. Arterial hypertension was the most frequent. Caution is needed in choosing treatment regarding comorbidities and the risk of infection in these patients.References:[1]Villa-Blanco JI, Calvo-Alén J. Elderly onset rheumatoid arthritis: differential diagnosis and choice of first-line and subsequent therapy. Drugs Aging. 2009;26(9):739-50.Disclosure of Interests:None declared

Background:The spondyloarthritis (SpA) family includes a heterogeneous spectre of inflammatory diseases with several common features, such as the axial and peripheral joint involvement, but also trigger factors, as for instance, mechanical stress, infectious diseases or gut dysbiosis.[1,2]Entheseal inflammation is the common background in the pathogenesis of SpA, leading to osteitis, periostitis and osteoproliferation. Despite being a central feature in SpA, enthesitis remains underdiagnosed in everyday practice.[3,4]Objectives:The primary objective is to assess the frequency of ultrasound versus clinically detected enthesitis in a sample of Romanian patients with highly active SpA. A secondary objective is to determine the existence of an enthesitis pattern according to the dominant side in this sample of patients.Methods:Out of 140 SpA patients, 106 were diagnosed with axial/peripheral SpA and 34 with psoriatic arthritis (PsA), and were clinically (medical history, clinical examination) evaluated, scanned using MSUS during the same visit, then asked which was their dominant side, in order to avoid any biases. The evaluation targeted 16 entheseal sites (Achilles tendon, plantar fascia, quadriceps tendon, proximal and distal patellar tendon, triceps tendon, extensor and flexor tendons of the hand, all evaluated on both sides), reaching a total of 2240 entheses. The medical history form included questions related to present or past spontaneous pain in any of the 16 evaluated sites as well as the clinical examination that evaluated the entheseal pain upon pressure (digital pressure on the enthesis overlying skin).The MSUS evaluations were conducted using Esaote My Lab machines with 6-12/8-18 MHz linear probes. The same clinician/ultrasonographer performed all evaluations, in order to avoid interobserver variability.Results:In the studied sample of patients, 68.6% were men, had a mean age of 43,46 (+/- 11,77), 51.4% were diagnosed with peripheral SpA, with a mean disease duration of 61,60 (+/- 71,03) months, and entheseal abnormalities were found in up to 62.7% of the asymptomatic entheses, lacking both spontaneous and elicited pressure pain.The best performance of clinical and ultrasound examination was observed in the evaluation of the flexor tendons of the hand, with strong agreement between the two methods (kappa = 0,718, p = 0.001). Conversely, the lowest performance of clinical and ultrasound examination was noticed in Achilles (kappa = 0,292, p = 0.001) and distal patellar tendons (kappa = 0,202, p = 0.001), with low agreement indices.Both GS and PD abnormalities were more frequently detected on the right side, in a sample of 98.57% right handed patients. The differences were higher regarding the insertions of the triceps tendon (2.2%) and the plantar fascia (2.1%), in favour of the dominant side.Conclusion:Entheseal reported pain (spontaneous and elicited by pressure) correlates poorly with MSUS detected enthesitis. This study highlights the high rates of imaging detected, but clinically overlooked entheseal abnormalities in SpA patients.Enthesitis was more frequently detected on the dominant side, emphasizing the role of mechanical stress in the pathogenesis of this feature. This outcome also requires the selection of the most reliable entheseal sites for SpA, being a future direction of research for this ongoing study.References:[1]Barnett R, Ingram T, Sengupta R. Axial spondyloarthritis 10 years on: still looking for the lost tribe. Rheumatology (Oxford). 2020; 59(4): iv25-iv37[2]Kehl AS, Corr M, Weisman MH, et al. Enthesitis - New Insights Into Pathogenesis, Diagnostic Modalities, and Treatment. Arthritis Rheumatol. 2016; 68(2): 312–322[3]Ruta S, Gutierrez M, Pena C, et al. Prevalence of subclinical enthesopathy in patients with spondyloarthropathy: an ultrasound study. J Clin Rheumatol. 2011; 17: 18–22[4]Yi E, Ahuja A, Rajput T, et al. Clinical, economic, and humanistic burden associated with delayed diagnosis of axial spondyloarthritis: a systematic review. Rheumatol Ther. 2020; 7: 65–87Disclosure of Interests:None declared.

Background:Sexual health is an essential element of overall health and well-being. Rheumatic diseases may affect sexual functioning in many ways related to pain, fatigue, stiffness, functional impairment, depression, anxiety, negative body image, reduced libido, hormonal imbalance and drug treatment. However, these issues are rarely addressed in clinical practice.Objectives:The aim of this study was to evaluate sexual function in a cohort of men with rheumatic disease compared to healthy controls.Methods:This was an observational, single-center, cohort study conducted between august 2019 and march 2020 in the Rheumatology department of “Saint Mary” Clinical Hospital in Bucharest which included 120 men with ages between 18 and 60 years - 60 patients with rheumatic diseases and 60 healthy controls. The study tools were the Sexual Health Inventory for Men (SHIM) questionnaire and one questionnaire referring to personal data, history of the rheumatic disease, comorbidities, treatment and sexual impairment. Also, the disease activity was assessed using specific scores for each condition.Results:In this cohort of 60 patients, the mean age was 45.26 (7.8) years and the diagnoses wereankylosing spondylitis (AS) - 37%,psoriatic arthritis (PsA) - 18%, rheumatoid arthritis (RA) - 17%, systemic sclerosis (SS) - 15% and gout - 13%. More than half of the patients (62%) had active disease based on specific scores (ASDAS for AS, DAS28-CRP for RA, EScSG disease activity indices for SS, DAPSA for PsA). Regarding sexual life, this study showed a significant decrease in sexual life quality after rheumatic disease diagnosis(before diagnosis: 71,67% - satisfying and 16,67% - not satisfyingversus after diagnosis: 21,67% - satisfying and 68,33% - not satisfying). Most patients (90%) reported impairment of their sexual life after diagnosis. In terms of sexual dysfunction (SD), a significantly higher proportion of patients (40%) mentioned reduced libido compared to the control group (18,33%) (p=0.043). Also, 21,66% of the patients reported erectile dysfunction (ED) in comparison with only 8,33% in the control group (p=0.009). Most patients with AS, RA, PsA and gout had mild ED while most patients with SS presented with mild to moderate ED. Also, the SHIM score mean value was significantly lower in the study group (17,65)compared to the control group (20,15) (p=0.009). The importance of SD in this cohort is emphasized by the fact that only one patient conceived after rheumatic disease diagnosis. Concerning treatment, more than half of the patients (55%) reported no effect of the therapy on their sexual life while 38.33% mentioned that medication improved their sexual life and very few (7%) reported a worsening.Conclusion:This study revealed a higher prevalence of sexual dysfunction in male patients with rheumatic disease in comparison with healthy controls. Considering the importance of sexual and reproductive health, rheumatologists should approach this topic with their patients and offer them guidance.References:[1]AG Tristano, “The impact of rheumatic diseases on sexual function”, Rheumatol Int 2009 Jun;29(8):853-60Disclosure of Interests:None declared

Background:Two pivotal studies, RAPIDS-1 and RAPIDS-2 revealed that bosentan reduces the development of new digital ulcers (DUs) in patients with systemic sclerosis (SSc). However data regarding the long-term use of this dual endothelin antagonist receptor in the treatment of DUs is scarce.Objectives:The aim of the present study was to evaluate long term efficacy and safety profile of bosentan in patients with DUs related to SSc.Methods:A prospective observational case-control study, conducted between 2014 and 2020 enrolled 65 SSc patients with ≥1 active DUs at baseline, who received bosentan therapy. Demographic and clinical features, including DUs incidence and patients subjective perception of DU pain and/or Raynaud’s Phenomenon, were collected. Nailfold videocapillaroscopy was performed in all patients.Results:The study included 51 females and 14 males, with a mean age of 52.6 years, 30 with diffuse subset, most of them with late scleroderma pattern (46/65). Number of DUs at baseline was 4.55 (±2.8), median duration of treatment was 25.95 (±19.4) months. Microangiopathy evolution score (MES) was 5.1 (2.19), visual analog scale (VAS) for DU was 77.9, VAS for Raynaud was 73.4.Patients receiving bosentan had clinically significant reduction in the mean number of DU (pThere was a clear trend towards an improvement in MES score, between baseline and the next follow-up assessments (p=0.003). The difference was statistically significant when compared to control group, but only for the first 18 months of treatment (p14 patients (28.75%) discontinued bosentan therapy for administrative reasons. The median time among patients who interrupted the treatment was 6.9 months. An accelerated development of new DU was described 6 months after (p=0.02). Following recommencement of bosentan, the mean number of DU has rapidly decreased (p=0.008). There was no significant difference between patients who temporarily discontinued bosentan for 6 or 12 months.Bosentan was stopped due to lack of efficacy in 2 cases and due to side effects in 7 cases: 4 elevated liver enzymes, 1 severe trombocytopenia, 1 dyspneea agravation and low blood pressure.Conclusion:The present data suggest that treatment with endothelin receptor antagonist bosentan was associated with a significant reduction in the mean number of DU in patients with SSc. The beneficial effect of bosentan persisted throughout the study but was most evident in the first 6 months of treatment. Statistical analysis showed a significant improvement of the microangiopathy evolution score from baseline to end of therapy. 14 patients had a high relapse rate due to potential rebound effect, 6 months after bosentan withdrawal.The drug was reintroduced succesfully for 10 (70%) patients with a significant decrease in the number of DU.References:[1]UK Scleroderma Study Group: digital vasculopathy in systemic sclerosis, Rheumatology, Volume 54, Issue 11, November 2015, Pages 2015[2]Groseanu L, Berghea F, Bojinca V, et al AB0779 Long term follow-up of a systemic sclerosis group treated with bosentan, Annals of the Rheumatic Diseases 2018;77:1523-1524.Disclosure of Interests:None declared

Background:Certainly, the year 2020 changed the healthcare system due to SARS-CoV2 pandemic that affected globally, more than 100 million people, causing more than 2 million of deaths worldwide. The evidence of how this infection impact patients with rheumatic and musculoskeletal diseases treated with disease modifying anti-rheumatic drugs is still an unmet need.Objectives:The main focus of this study is to evaluate the influence of DMARDs therapy on the evolution of COVID-19 disease in patients with RMDs. The second objective is to study and find correlations between the severity of infection in patients with rheumatic diseases.Methods:A retrospective observational study was conducted between June 2020 and January 2021, enrolling 81 patients with rheumatic diseases that went through SARS-CoV2 infection. The data was collected using patients’ clinical documents and through telemedicine, in accordance with EULAR COVID-19 Rheumatological Database.Results:Among the 81 patients, 53 (65,43%) were females and 28 (34,56%) were males. The mean age was 47,9 years old (49,49 years old for females and 45,25 years old for males). The majority lives in urban areas – 62 patients (76,54%).The temporal trends of COVID-19 observed in this cohort was in consonance with the evolution of the pandemic in Romania: one third of cases were recorded between June and October 2020 and two-thirds between November 2020 and January 2021, when the number of COVID-19 cases tripled in the general population.Surprisingly, more than 27% of patients in this study were asymptomatic at the time of COVID-19 diagnosis. They were tested according to the protocol before admission to the hospital. 9,8% of patients also asymptomatic, were tested positive as a screening before leaving the country. The majority (45,6%) were symptomatic or contact with someone infected with SARS-CoV2-and tested positive with RT-PCR.We divided the cohort in 3 groups: patients with mild infection that required no hospitalization (22 patients counting for 27,16%), moderate infection – hospitalization but not in the Intensive Care Unit (52 patients – 64,19%) and severe infection – admission to the ICU/deaths (7 patients in the ICU, 4 deaths – 4,9%).Mild and moderate COVID 19 disease was identified in patients with axial spondyloarthtis (56,7%), with remission or with low disease activity, with a few or no comorbidities, with a mean age of 47,56 years old and also in patients in treatment with MTX (14,86%) or TNF alfa inhibitors (35,13%). 51% of patients stopped the therapy during COVID19 diseases.Factors correlated with severe infection and death were age (the mean age was 62,14), high and moderate disease activity RA, overlap syndromes (RA with SLE or Sjogren Syndrome) and important cardiovascular comorbidities. Two of the deceased patients were in treatment with MTX and RTX (the last infusion was more than 6 months).Conclusion:The data in our study suggests that the use of cs DMARDs (MTX) and TNF alfa inhibitors is associated with better outcomes for patients with RMDs and COVID-19. These results are in accordance with the data found in literature [1,2,3]. The limitation of this study is the little number of patients and the fact that the real number of COVID-19 cases might be higher in reality due to asymptomatic or pauci-symptomatic patients.References:[1]Filière des Maladies Autoimmunes et Autoinflammatoires Rares (FAI2R); Hôpital Huriez, CHU Lille, Univ. Lille, Lille, France, Severity of COVID-19 and survival in patients with rheumatic and inflammatory diseases: data from the French RMD COVID-19 cohort of 694 patients, Annals of the Rheumatic Diseases Published Online First: 02 December 2020[2]Sanchez-Piedra C et al., On behalf of the BIOBADASER study group, et al, Clinical features and outcomes of COVID-19 in patients with rheumatic diseases treated with biological and synthetic targeted therapies,Annals of the Rheumatic Diseases 2020;79:988-990.[3]Hyrich, K.L et al. Rheumatic disease and COVID-19: epidemiology and outcomes. Nat Rev Rheumatol 17, 71–72 (2021)Disclosure of Interests:None declared

Background:The low overall prevalence of systemic sclerosis (SSc) and the low proportion of male patients have resulted in a scarcity of studies assessing sex differences in SSc patients, and contradictory results have often been observed.Objectives:The aim of the study was to assess differences in disease manifestations in a cohort of SSc patients according to gender.Methods:We performed a retrospective observational study using data extract from the EULAR scleroderma trials and research (EUSTAR) cohort 096.We looked at sex influence on disease characteristics at baseline and then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival.Results:173 patients with SSc were available for the baseline analyses. Males were older (52,96 vs 45,88, p=0.009), were more likely to smoke (73% vs 7%, pIn the longitudinal analysis after a mean follow-up of 3,5(±0,65) years, male sex was associated with a higher risk of scleroderma renal crisis (OR:9.45 (1.49 to 59.69); p=0.004), digital contractures (OR:8,2 (3,1 to 21,9); pConclusion:Although more common in women, SSc appears as strikingly more severe in men. Our results demonstrate a higher risk of severe organ involvement and poor prognosis in men. These results raise the point of including sex in the management and the decision-making process.Disclosure of Interests:None declared

Background:Since the beginning of 2020, the medical staff caring for patients with rheumatic disorders has been exposed to considerable emotional and physical stress due to the COVID19 pandemic outbreak. Newly-designed circuits were imposed to better manage chronic patient admissions.Objectives:To assess the level of motivation in healthcare personnel caring for patients with rheumatic disorders and identify determinants that lead to dissatisfaction during the COVID19 spread.Methods:A 15-item questionnaire was distributed among medical and paramedical staff looking after chronic rheumatic patients. Motivation factors were included in questions having five preset answers, ranging from “not al all”, “to a small extent”, “moderately”, “to a large extent” and “to a very large extent”. The final item was an open-type question, favoring personal suggestions and potential changes in local management.Results:Out of a total of a 124 responders, 82 were doctors with various degrees in training (fellow trainees, specialists in Rheumatology, Head of Department), 37 medical assistants, 3 nurses, a physical therapist, one clinical psychologist. Mean age in the group was 38.3 years old with a strikingly female dominance (87.6%). Within the responders, the financial factor represented by monthly salary or bonuses had no or little impact on work motivation during the pandemic since more than 70% answered with “not al all / to a small extent / moderately”. Regarding motivation through working hours, 26.7% responded either “not at all” or “to a small extent”, 32.4% were moderately motivated while only 12.4% were more enthusiastic. More than half (53.4%) of the staff stated that motivation was not entirely affected by working conditions in the hospital. Answers were relatively equally divided referring to motivation by safety at their workplace (almost 20% for each statement), and 40.9% suffered some extent of demotivation due to risk of contamination while caring for COVID19 positive patients. 42% of the staff was only moderately motivated by hospital’s measure to limit the spread of COVID throughout the personnel. Almost 75% of the healthcare workers were not motivated by news media. Around 54% said that personal motivation was not influenced by a larger work volume and 43% were very much motivated by work relationships with colleagues. 41% of the staff is only moderately satisfied with their work during the COVID outbreak while 18% are not at all satisfied. Finally, the last open-question confirmed that for some the pandemic period was a time to bring healthcare professionals together to a stronger bond. Other responders felt that chronic rheumatic patients were left aside, while caring mostly for COVID19 patients and only limited admissions for life-threatening conditions. Moreover, medical staff suggested that the lack of a more frequent testing throughout the personnel to avoid contamination was demotivating, while still living with families or relatives.Conclusion:Motivation of the personnel is a process with great economic and social impact. This study challenged the level of healthcare staff in difficult times caused by medical crisis. Results of the enquiry showed that financial benefits had no impact on motivation. Harsher working conditions or larger volume did not negatively impact motivation of staff, but the fear of contamination was considerable. Apparently the media influence was not a motivator for medical staff during the outbreak. Medical teams should find their stamina until the end of pandemia.References:[1]Barati, M, Bashirian, S et al, Factors associated with preventive behaviours of COVID-19 among hospital staff in Iran in 2020: an application of the Protection Motivation Theory, Journal of Hospital Infection 2020.[2]Barati, M, Bashirian, S et al, Factors associated with preventive behaviours of COVID-19 among hospital staff in Iran in 2020: an application of the Protection Motivation Theory, Journal of Hospital Infection 2020.Disclosure of Interests:None declared

Background:Treatment with low-dose glucocorticoids (GCs) (≤7.5 mg prednisolone) in combination with standard care is highly effective in rheumatoid arthritis (RA), but despite 70 years of clinical experience, evidence-based information on its balance of benefit and harm is incomplete. This leads to an ongoing debate, with under- and over-use of GCs as result. The GLORIA pragmatic trial was developed to assess harm, benefit and costs of low-dose GCs added to the standard treatment of older RA patients.Objectives:The objective of this abstract is to document the baseline status and frequency of comorbid conditions in the GLORIA study population. The results of the unblinded data will be submitted as late-breaking abstract.Methods:This double-blind, randomized, placebo-controlled, multicenter trial (1) was open for patients with RA according to the 1987 or 2010 (2) criteria, age ≥65 years, and disease activity score of 28 joints (DAS28) of ≥2.6. Patients were recruited from rheumatology clinics in Germany, Hungary, Italy, The Netherlands, Portugal, Romania and Slovakia. Eligible patients were randomized to two years of treatment with daily 5 mg prednisolone or matching placebo. All other medication was allowed, except for GCs. The presented data are blinded because the database is not closed yet.Results:The population consists of 451 patients with mean disease duration 10.6 (Q1-Q3: 3-15) years. The majority (70%) is female, mean age is 72.5 (Q1-Q3: 68-76, range: 65-88) years, 66% were positive for rheumatoid factor and 56% for ACPA. Patients had a mean of 4.3 (SD 2.8) comorbidities besides RA (3.4 active) and therefore used multiple concomitant medications (3.9 (SD 3.4)) (Table 1). The most common comorbidities (provisional data of 161 patients with complete coding) in this older population are: vascular disorders (58%), musculoskeletal and connective tissue disorders (57%) and a history of surgical and medical procedures (45%). Patients were most frequently on beta blocking agents (22%, mainly metoprolol) and HMG CoA reductase inhibitors (20%, mainly simvastatin). Most patients also have an extensive history of anti-rheumatic treatment. At the start of the trial most patients (82%) were on cDMARD treatment; 15% were on bDMARDs/tsDMARDs. Almost half of the patients previously had been treated with GCs, with a mean duration of 3.4 years and a mean last dose of 4.6 mg/day.Conclusion:The baseline data shows that we have an older study population who have relatively many other comorbidities next to RA and who are almost all treated with multiple concomitant medications in addition to the study medication. Therefore, we expect to report a high adverse event rate. Research among older patients is urgently needed, but the frailty of this population as represented by the multiple comorbidities and concomitant medications have to be taken into account in the analyses and interpretation of the results.References:[1]Hartman L, Rasch LA, Klausch T, Bijlsma HWJ, Christensen R, Smulders YM, et al. Harm, benefit and costs associated with low-dose glucocorticoids added to the treatment strategies for rheumatoid arthritis in elderly patients (GLORIA trial): study protocol for a randomised controlled trial. Trials. 2018;19:67.[2]Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO, et al. 2010 Rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheum. 2010;62:2569-81.Table 1.Comorbidities and concomitant medications at baseline in the GLORIA trial.MeanSDRangeComorbidities 4.32.8 0-15 Active 3.4 Past 1.9Concomitant medications (count) 3.93.4 0-15 Beta blocking agents (%)22 HMG CoA reductase inhibitors (%)20 Platelet aggregation inhibitors (%)16 ACE inhibitors (%)12 Angiotensin II antagonists (%)11DAS28 4.521.05DAS28CRP 4.060.97HAQ (0-3) 1.20.7RA treatmentCurrent (%)Previous (%) cDMARD8492 bDMARD/tsDMARD1522 NSAID5129 Glucocorticoids 049Acknowledgements:The GLORIA project is funded by the European Union’s Horizon 2020 research and innovation programme under the topic ‘’Personalizing Health and Care’’, grant agreement No 634886.Disclosure of Interests:None declared

Objective. The purpose of the present study was to assess the relevance of therapeutic drug monitoring in spondyloarthritis patients, by determining drug serum levels and anti-drug antibodies and estimating cut-off values for three TNF inhibitors. Methods. Over one year, we enrolled 100 patients with SpA, under consequent treatment with adalimumab (ADL), etanercept (ETA) or infliximab (IFX). Demographic, clinical (BASDAI, ASDAS) and laboratory (ESR, CRP) data was collected together with drug serum level and anti-drug antibodies using the ELISA technique. The statistical analysis was performed using the SPSS software, version 20.0 with the aid of Student t-test, Spearman and Pearson tests. Results. Out of the study cohort, 35% were on ADL, 33% on IFX, and 32% under ETA treatment. Undetectable drug levels correlated to the presence of anti-drug antibodies and to disease activity scores. There were no identified anti-ETA antibodies. For this study lot trough levels are estimated between 2 and 4 μg/mL for an ASDAS-CRP under 2.1. Conclusion. Serum drug level measurement and anti-drug antibody detection can be used as a completion to a clinician’s tools in assessing disease activity, leading to an optimal and personalized manner of patient management.

Sjögren syndrome (SS) is a chronic autoimmune condition that mainly targets exocrine glands where it produces lymphocytic infiltrates. The clinical display varies in severity and can include malignancy association such as non-Hodgkin lymphoma. Its incidence ranges from 5 to 8%, while the risk of occurrence is 16 to 44 times higher. This article aims to describe the evolution of a patient with primary SS who developed pulmonary lymphoma for which she received Rituximab as part of the oncological therapeutic scheme. During the course of the disease she developed membranous glomerulonephritis, rarely described in these patients and low complement levels cited as predictive factor for lymphoproliferation.

Background:Adherence is a serious problem in treatment of inflammatory diseases. To measure adherence, caps that record medication bottle openings may be superior to capsule counts (1). In the ongoing two-year GLORIA trial on the addition of low-dose (5 mg) prednisolone or placebo to standard of care in elderly patients (65+ years) with rheumatoid arthritis, adherence was measured in both ways during the whole trial.Objectives:To describe adherence patterns, and to compare adherence as assessed with adherence caps and with capsule counts in the GLORIA trial.Methods:The recorded adherence patterns of patients (blinded for treatment group) were classified according to descriptive categories. Overall adherence according to number of bottle openings was compared with adherence according to the capsule count. Good adherence was defined as 80%: i.e. for caps 80% of days one opening recorded, and for counts less than 20% of prescribed tablets returned at the subsequent visit. Each patient has a maximum of 8 periods of 90 days.Results:Trial inclusion has closed in 2018 at 452 patients; the current dataset contains adherence data of 385 patients. Mean number of recorded 90-day periods per patient was 4 (range 1-8). Based on capsule counts over all periods, 90% of the patients met the 80% threshold of adherence; based on cap data only 31% met this criterion.The four adherence patterns are shown in a calendar matrix, with yellow for zero, green for one and blue for ≥two openings on a day (Figure 1). Bottles were supposed to be opened once a day.Patients were categorized according to the opening pattern seen in at least 50% of assessed periods:32% non-use(26% stable use(≥80% of the days 1 opening);40% irregular use(different adherence patterns, in or between periods);2% weekly use(1 opening per week).Conclusion:In our trial of elderly rheumatoid arthritis patients, patients appeared to be mostly adherent according to conventional capsule counts. Results from adherence caps were highly discrepant with the capsule counts, with patterns suggesting patients did not use the bottle for daily dispensing, despite specific advice to do so.References:[1] El Alili M, Vrijens B, Demonceau J, Evers SM, Hiligsmann M. A scoping review of studies comparing the medication event monitoring system (MEMS) with alternative methods for measuring medication adherence. Br J Clin Pharmacol 2016;82:268-79.Acknowledgments:The GLORIA project is funded by the European Union’s Horizon 2020 research and innovation programme under the topic ‘’Personalizing Health and Care’’, grant agreement No 634886.Disclosure of Interests:Linda Hartman: None declared, Sabrina Paolino: None declared, Reinhard Bos: None declared, Daniela Opris-Belinski Speakers bureau: as declared, Marc R Kok Grant/research support from: BMS and Novartis, Consultant of: Novartis and Galapagos, Hanneke Griep-Wentink: None declared, Ruth Klaasen: None declared, Cornelia Allaart: None declared, George Bruyn: None declared, Hennie Raterman Grant/research support from: UCB, Consultant of: Abbvie, Amgen, Bristol-Myers Sqibb, Cellgene and Sanofi Genzyme, Marieke Voshaar Grant/research support from: part of phd research, Speakers bureau: conducting a workshop (Pfizer), Nuno Gomes: None declared, Rui Pinto: None declared, Thomas Klausch: None declared, WIllem Lems Grant/research support from: Pfizer, Consultant of: Lilly, Pfizer, Maarten Boers: None declared

Background:The low overall prevalence of systemic sclerosis (SSc) and the low proportion of male patients have resulted in a scarcity of studies assessing sex differences in SSc patients, and contradictory results.Objectives:To evaluated sex influence on disease characteristics at baseline and then to estimate the effects of sex on disease progression and survival.Methods:We performed a retrospective observational study using data extract from the EULAR scleroderma trials and research (EUSTAR) cohort 096. 173 patients were analysed (26 males).The severity of organ system involvement was defined as described previously (1).Results:Males were significantly older at symptom onset (p=0.007) and at first center visit (p=0.009). There were no differences regarding disease duration at first visit or the interval between the onset of Raynaud syndrome and other non-Raynaud manifestations (p=0.06). Male patients were significantly more likely to have ever smoked (pIn multivariate analysis, male sex was independently associated with a higher risk of diffuse cutaneous subtype (OR: 1.56, (1.35 to 1.84); pConclusion:In essence, the disease prophyle in females is that of younger age of onset, longer disease duration at first center visit, less severe peripheral vascular involvement, the most frequent cause of death being PAH. In contrast, males are older at onset, present earlier in their disease, have dcSSc, more severe peripheral vascular disease, higher mRSS, more frequent and severe ILD, more frequent heart involvement, higher risk of PAH and SRC, the most common cause of death being ILD. These results raise the point of including sex in the management and the decision-making process.References:[1]Peoples C, Medsger TA Jr, Lucas M et al Gender differences in systemic sclerosis: relationship to clinical features, serologic status and outcomes.J Scleroderma Relat Disord. 2016;1(2):177–240Disclosure of Interests:Laura Groseanu Speakers bureau: novartis, eli-lilly, ucb, pfizer,sandoz, Andra Balanescu Consultant of: pfizer, Speakers bureau: Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, UCB, Violeta Bojinca Speakers bureau: Eli-Lilly, Novartis, Pfizer, Daniela Opris-Belinski Speakers bureau: Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Ioana Saulescu Speakers bureau: Eli-Lilly, Pfizer, Diana Mazilu: None declared, Sanziana Daia-Iliescu Speakers bureau: sandoz, Andreea Borangiu: None declared, Florian Berghea Paid instructor for: abbvie, Speakers bureau: gideon richter, egis, novartis,ucb, cosmin-laurentiu constantinescu: None declared, CLAUDIA COBILINSCHI Speakers bureau: novartis, Maria Magdalena Negru: None declared, mihai abobului Speakers bureau: gideon richter, Ruxandra Ionescu Consultant of: Consulting fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Speakers bureau: Consulting and speaker fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz

Background:Echographic evaluation of salivary gland increasingly became a routine imaging modality in patients with Sjogren’s syndrome (SS). However, predictive parameters associated with the severity of echographic features are still incomplete evaluated.Objectives:The aim of this study was to evaluate the predictors for severe echographic involvement in patients with SS followed in a tertiary center.Methods:We included 63 patients with SS (mean age: 52.3±11.9; 59 female). The complete laboratory workup, clinical manifestations and treatment were reviewed and the EULAR Sjögren’s syndrome disease activity index (ESSDAI) was calculated for each patients. We performed the standard echographic evaluation of salivary gland in all patients and used a 4 grade system for severity staging.Results:The distribution of echographic grade was: no any echographic features – 4 pts (6.3%); grade 1 - 24 pts (38.1%); grade 2 – 20 pts (31.7%); grade 3 – 10 pts (15.9%); grade 4 – 5 pts (7.9%). The ESSDAI and the hydroxichloroquine use were similar in these subgroups. We didn’t find differences regarding CRP and fibrinogen and echographic features. The age of the patients, the anti-SSA and anti-SSB, ESR, total protein, IgA, IgG and rheumatoid factor levels were significantly higher and lymphocyte count was lower in patients with echographic severity above grade 2 when compared with patients with no or mild echographic features. However, using ANOVA test and post-hoc analysis, the only parameters associated with the severity of echographic features were high ESR (53 vs 17 in grade 4 vs 1, p=0.02), IgA (363 vs 190 in grade 4 vs 1, p=0.004) and IgG (1985 vs 1191 U/l in grade 4 vs 1, p=0.001) levels.Conclusion:Parameters linked to polyclonal hypergammaglobulinemia (IgA and IgG levels; and ESR) seem to be linked to the severity of echographic appearance of salivary gland in patients with SS. Further studies are needed in order to better characterize this link.Disclosure of Interests:Ancuta MIHAI: None declared, DENISE MARDALE: None declared, Daniela Opris-Belinski Speakers bureau: as declared, Ruxandra Ionescu Consultant of: Consulting fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Speakers bureau: Consulting and speaker fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Ciprian Jurcut: None declared

Background:Medication adherence in the GLORIA trial, among elderly patients with rheumatoid arthritis, is measured with caps that register openings of the medication bottle. At each study visit, one or two medication bottles with cap (kits) are dispensed, each containing 90 capsules. Multiple steps are needed to come to a workable dataset to describe adherence.Objectives:To describe the steps that are needed to come from raw data to a workable dataset to analyze adherence data that are recorded by electronic caps.Methods:The medication bottle contains a cap with the ability to register cap openings. The raw dataset from the caps consist of an excel file with one opening event per row, recorded as date and time. One cap yields approximately 90 rows. First, the kit numbers were matched to the corresponding patient numbers, that are recorded in another excel file. Instances where two kits were dispensed were recorded with two kit numbers in one cell and need to be copied to two cells with one kit number. Second, the VLOOKUP function was used to combine dates and kit numbers. One row now contains all openings from one kit. Then, the number of days between first opening and each next opening date was calculated. A range of 90 days was made to calculate how many times the bottle was opened on each day of the 90-days period. The results were color-coded to visualize instances of zero, one or ≥two openings on a day.Results:The colored calendar matrix (Figure 1) can now be used to categorize adherence patterns.Conclusion:A monitoring cap seems a simple instrument to measure adherence. However, multiple steps and a lot of time are needed to come to a workable dataset for the study of adherence patterns.Acknowledgments:The GLORIA project is funded by the European Union’s Horizon 2020 research and innovation programme under the topic ‟Personalizing Health and Care’’, grant agreement No 634886.Disclosure of Interests:Linda Hartman: None declared, Elisa Alessandri: None declared, Reinhard Bos: None declared, Daniela Opris-Belinski Speakers bureau: as declared, Marc R Kok Grant/research support from: BMS and Novartis, Consultant of: Novartis and Galapagos, Hanneke Griep-Wentink: None declared, Ruth Klaasen: None declared, Cornelia Allaart: None declared, George Bruyn: None declared, Hennie Raterman Grant/research support from: UCB, Consultant of: Abbvie, Amgen, Bristol-Myers Sqibb, Cellgene and Sanofi Genzyme, Marieke Voshaar Grant/research support from: part of phd research, Speakers bureau: conducting a workshop (Pfizer), Nuno Gomes: None declared, Rui Pinto: None declared, Thomas Klausch: None declared, WIllem Lems Grant/research support from: Pfizer, Consultant of: Lilly, Pfizer, Maarten Boers: None declared

Background:Various cutaneous manifestations are described in patients with Sjogren’s syndrome (SS). However, the predictors for its occurrence are not fully investigated. Recently, data regarding the role of hematological parameters ratios in patients with rheumatic diseases were reported. However, routine hematological parameters and the derived ratios were not studied in SS patients.Objectives:The aim of this study was to evaluate the predictors for cutaneous manifestations in a large cohort of patients with SS followed in a tertiary center.Methods:We included 114 patients with SS (mean age: 53.1±12.1; 94.7% female). We divided the patients in group A – patients with cutaneous manifestations and group B – without cutaneous manifestations. The complete laboratory workup [including the calculation of neutrophil to lymphocyte ratio (NLR), mean platelet volume (MPV) to platelet ratio, platelet distribution width (PDW) to platelet ratio], clinical manifestations and treatment were reviewed and the EULAR Sjögren’s syndrome disease activity index (ESSDAI) was calculated for each patients.Results:Cutaneous manifestations were diagnosed in 11 patients (9.6%). The age of the patients, the levels of anti-SSA, complement, inflammatory markers (ESR, CRP), immunoglobulins and vitamin D levels, the ultrasonographic score of salivary glands and the ESSDAI value were similar in both groups. The patients with cutaneous manifestations were more frequently treated with hydroxychloroquine (9/11 vs 49/103, p=0.03) but the mean doses were similar in both groups. The values of anti-SSB were marginally higher in patients with cutaneous manifestations (102.3±83.1 vs 53.6±79.9, p=0.07). The lymphocyte, neutrophil count, MPV and PDW were similar in both groups, while platelet count was lower in patients with cutaneous manifestations (224090.9±59528.9 vs 264339.8±60669.0, p=0.03). The MPV/Plt ratio tended to be higher in patients with cutaneous manifestations (0.051±0.017 vs. 0.043±0.013, p=0.07), but the values of PDW/Plt ratio (0.064±0.022 vs 0.051±0.018, p=0.03) and of NLR (3.82±2.30 vs 2.35±0.92, p=0.001) were found to be significantly higher in these patients. In multivariate analysis, only the NLR remains independently associated with cutaneous manifestations (p=0.001). Using the ROC curve analysis, a cutoff of 2.70 for NLR had a 63% sensitivity and 69% specificity for cutaneous manifestations.Conclusion:The hematological parameters related to platelets appear to be modified in patients with SS and cutaneous manifestations. However, only neutrophil to lymphocite ratio is independently associated with cutaneous involvement in these patients.Disclosure of Interests:Ancuta MIHAI: None declared, DENISE MARDALE: None declared, Daniela Opris-Belinski Speakers bureau: as declared, Ruxandra Ionescu Consultant of: Consulting fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Speakers bureau: Consulting and speaker fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Ciprian Jurcut: None declared

Background Patients with ankylosing spondylitis (AS) are substantial users of healthcare resources due to chronic and potentially disabling disease. This study assessed the impact of adalimumab on clinical outcomes, healthcare resource utilization, and sick leave in patients with AS in five Central and Eastern Europe (CEE) countries. Methods This was an observational study in the routine clinical setting. Patients diagnosed with AS and starting treatment with originator adalimumab were followed for 12 months by assessing disease activity (Bath Ankylosing Spondylitis Disease Activity Index [BASDAI] and Ankylosing Spondylitis Disease Activity Score [ASDAS]) and physical function (Bath Ankylosing Spondylitis Functional Index [BASFI]). Data on healthcare resource utilization and sick leave were collected prospectively and compared with historical data before adalimumab initiation, as well as between treatment responders and non-responders defined by BASDAI-50. Results The total effectiveness population comprised 450 patients with on average long-standing AS, high disease activity, and functional impairment. At 12 months of adalimumab therapy, mean ASDAS and BASFI scores were in the range of low disease activity and normal physical function, respectively. The mean number of hospital admissions, hospital inpatient days, and healthcare provider visits were decreased by 67.9, 83.0, and 46.3%, respectively. The number and length of sick leaves were decreased by 65.6 and 81.4%, respectively. Reductions were higher in treatment responders than non-responders. Conclusion Originator adalimumab in routine clinical practice in five CEE countries produced clinically meaningful improvements in disease activity and physical function, and it was associated with reductions in healthcare resource utilization and sick leave.

Background GLORIA is an ongoing large pragmatic trial that examines harm, benefit and costs of low-dose glucocorticoids added to the standard treatment of RA patients of 65 years or older. The eligibility criteria are non-restrictive: RA, age ≥65 years, disease activity score (DAS28) of ≥2.6, and no current glucocorticoid treatment. Patients with comorbidity are expressly included, and the impact of trial procedures on normal care is minimal. Nevertheless, inclusion proves to be challenging. We have prospectively sampled all the reasons for ineligibility across a number of centres in different countries participating in the GLORIA trial. Methods Rheumatologists from 8 centres in Germany, Hungary, The Netherlands, Portugal and Romania screened the patient list of at least two full clinic days. For each patient, the eligibility and all possible reasons of exclusion were recorded. Results In total, 385 patients were screened. Of these patients, 15 (4%) were eligible to participate in the GLORIA trial. In Germany, Romania and Portugal (Lisbon) none of the screened patients proved eligible. The most common reasons for ineligibility were inactive disease and age (both 58%) (table 1). Current glucocorticoid use was reported in 28%, 5% had a temporary reason (i.e. recent switch of therapy or glucocorticoid use), and 51% had more than one reason for ineligibility. We found remarkable differences between the sites in the distribution of the main reasons for ineligibility (table 1). Of the eligible patients, 1 was already participating, 3 were included after this screening, and 2 were currently considering participation; 9 declined participation (most common reasons: fear of glucocorticoids, not interested to participate, preference for GC injections or declining additional therapy). Conclusions In this prospective study, we found remarkable differences between countries in reasons for non-participation in our ongoing GLORIA trial. The willingness of eligible patients to participate was low in this elderly population, despite the pragmatic design. Earlier studies also showed that it is challenging to include elderly patients in a clinical trial.1 2 Pre-screening of patients in potential sites can provide important information on the potential to recruit patients in a trial, but the actual willingness of patients to participate remains hard to predict. References [1] Calamia M, et al. I’d Do Anything for Research, But I Won’t Do That: Interest in Pharmacological Interventions in Older Adults Enrolled in a Longitudinal Aging Study. PLoS One2016;11:e0159664. [2] Denson AC, et al. Participation of the elderly population in clinical trials: barriers and solutions. Cancer Control2014;21:209–14. Acknowledgements This project has received funding from the European Union’s Horizon 2020 research and innovation programme under grant agreement No 6 34 886. Disclosure of Interest None declared

Background In patients with primary Sjogren Syndrome (pSS), haematological involvement – autoimmune cytopenia, might be present at the time of the diagnosis or can develop in time after the characteristic glandular involvement. (1,2) Objectives The objective of the study is to evaluate the correlation between glandular involvement (ocular) and presence of cytopenia in patients diagnosed with pSS. Methods A retrospective analysis was performed on a cohort of patients diagnosed with primary Sjogren Syndrome under surveillance in one Rheumatology Centre between 2009 and 2016. The documented cases have been diagnosed according to the 2002 American-European Consensus group classification criteria, the 2012 ACR criteria or 2016 ACR/EULAR Classification Criteria for pSS. The EULAR Sjogren’s Syndrome Disease Activity Index (ESSDAI) was calculated for all patients. Ocular assessment and follow-up were performed in collaboration with the same ophthalmologist. The data was analysed using Windows Excel/SPSS20.0. Results 30 female patients diagnosed with pSS were included in the study. The mean age at the time of diagnosis was 52.1 years±SD 9.1. The ESSDAI was calculated for all patients at baseline: 5 (17%) patients presented high disease activity (ESSDAI >14), 14 (46%) patients moderate disease activity (5≤ESSDAI≤13) and 11 (37%) patients low disease activity (ESSDAI In the clinical case series, Spearman’s rank correlation coefficient between haematological (autoimmune cytopenia), and biological markers (hypocomplementemia) and ocular involvement were calculated. A strong negative correlation was found between autoimmune cytopenia and glandular manifestations (ocular involvement-xerophthalmia) (r=-0,60; p Conclusions Patients diagnosed with primary Sjogren Syndrome that presented at disease’s onset cytopenia and hypocomplementemia had a less severe ocular involvement. References [1] Seror R, Theander E, Brun JG, et al, Validation of EULAR primary Sjogren’s syndrome disease activity (ESSDAI) and patient indexes (ESSPRI)Annals of the Rheumatic Diseases 2015;74:859–866. [2] Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ, Mariette X, International Sjogren’s Syndrome Criteria Working Group, Ann Rheum Dis. 2017Jan;76(1):9–16. [3] Koh JH, Lee J, Chung SH, Kwok SK, Park SH, Relation of Autoimmune Cytopenia to Glandular and Systemic Manifestations in Primary Sjogren Syndrome: Analysis of 113 Korean Patients, J Rheumatol. 2015Oct;42(10):1817–24. Disclosure of Interest None declared

Background Prospective studies with Bosentan have shown short term efficacy, while it is not clear whether long-term treatment may be effective or whether ulcers may recur once treatment is discontinued. Objectives Our objective was to evaluate the long term efficacy and tolerability of bosentan in patients with systemic sclerosis (SSc) who develop digital ulcers (DU). Methods In the present prospective, observational, non-controlled study, we followed 26 SSc patients treated with Bosentan from Sept 2014 to Dec 2017 Number of DU, semiquantitative capillaroscopic scoring, VAS (visual analoque scale) for Raynaud, VAS for DU and HAQ were evaluated every 6 month. Results are presented as mean(sd). The difference between efficacy measures at follow-up visits was tested with the Wilcoxon’s signed-rank test. Results The group included 26 patients, 16 females, 11 diffuse subsets, age was 48.08 (9.8) years, disease duration was 84.35 (76.04) months, number of DU was 4.27 (3.71), most of them had a late scleroderma pattern pattern (16/26). Microangiopathy evolution score was 5.19 (2.04), VAS for DU was 75.52 (16.17), VAS for Raynaud was 67,43 (14.16), HAQ was 1.62 (0.55). 5 patients received Bosentan less then 6 month, so they were excluded from the statistical analysis. 6 month evaluation revelead significant decrease in the number of DU (p Regarding Bosentan safety: 6 patients died during the follow up (3 cases of severe pulmonary arterial hypertension, 1 scleroderma renal crisis, 1 heart failure, 1 post vascular surgery). Bosentan was stopped due to lack of efficacy in 2 case and due to side effects in 3 cases: 2 elevated liver enzymes, 1 severe trombocytopenia and 1 dyspneea agravation. 12 patients had a follow up after a 6 months Bosentan stop. We did not notice any significant increase in the number of DU, the VAS for DU or Raynaud, the capillaroscopic semiquantitative scoring or the HAQ. Conclusions We noted a significant decrease in the number of DU, patients perception of Raynaud and of DU after 6 months of treatment and the effect was maintained in the 3 years follow-up, even 6 months after Bosentan was stopped. In this long-term follow-up no new unidentified adverse reactions were found, except for the unexpected severe thrombocytopenia. The present study is limited due to the small sample size, to the observational nature and should be viewed as descriptive. Questions rise about drug costs (6 months or long term treatment), but it also has to be emphasised that most of these lesions were chronic and nonresponsive to previous treatments. References [1] Matucci-Cerinic M, et al.Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis. 2011;70(1):32–8 2] P. Garcia de la Pena-Lefebvre, et al. Long-term experience of bosentan for treating ulcers and healed ulcers in systemic sclerosis patients. Rheumatology (Oxford). 2008;47(4):464–6. Disclosure of Interest None declared

Background There is limited data available (case series, small clinical trials and expert opinion) regarding the need to initiate immunosuppressive therapy in patients with primary Sjogren syndrome (pSS). Objectives The aim of this study is to determine the factors that correlate with physician’s decision to start immunosuppressive therapy in pSS patients. Methods Subjects with pSS diagnosed according to the classification criteria in use at the time of their first presentation, were included in a monocentric cohort. A retrospective analysis was performed. The EULAR Sjogren’s Syndrome Disease Activity Index (ESSDAI) at onset and Sjogren Syndrome Damage Index(SSDI) at the last evaluation were calculated. Treatment was given according to the physician’s decision. Laboratory tests and Ultrasonography(US) of major salivary glands were performed in all cases. The data was analysed using Windows Excel/SPSS20.0. Results Corticotherapy was prescribed in 26/30 cases (86.6%), mean duration 50.65 months. Immunomodulatory treatment with hydroxychloroquine was given in 26/30 cases (86.6%). Immunosuppressive treatment was required in 10/30 patients (33.3%)- azathioprine 7 (23.3%) cases, methotrexate 3 (10%)cases. The mean ESSDAI score was 6.83±SD 1.8. In 19 (63.3%)cases disease activity was moderate or high (ESSDAI >5). The mean damage score value (SSDI) was 3.1±SD1.2. There was a moderate correlation between the activity score ESSDAI and the damage score SDDI (r=0.41, p Conclusions An important number of patients received corticotherapy, immunomodulatory agents and immunosuppressive therapy. The decision to initiate and maintain immunosuppressive therapy correlated with hypergammaglobulinemia and specific Sjogren’s US changes. The damage score(SSDI) does not correlate with immunosuppressive therapy duration. References [1] Rischmueller M, Tieu J, Lester S. Primary Sjogren’s syndrome. Best Pract Res Clin Rheumatol2016Feb 30. [2] Seror R, Bootsma H, Saraux A, et al. Defining disease activity states and clinically meaningful improvement in primary Sjogren’s syndrome with EULAR primary Sjogren’s syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI). Ann Rheum Dis2016. Disclosure of Interest None declared

Background While macrovascular disease and higher cardiovascular (CV) risk are well documented in other systemic rheumatic diseases, the risk for major cardiovascular events for patients with systemic sclerosis (SSc) is yet to be established. Objectives The aim of the study was to determine the ability of different cardiovascular risk indices to predict major cardiovascular events (MACE) in systemic sclerosis. Methods The study included 144 patients followed in EUSTAR centre 096, but only patients with a follow-up for more then 10 years were selected for statistical analyses. Cardiovascular risk was estimated using QRiskII, systemic coronary risk evaluation (SCORE) and ACC/AHA risk indices. MACE were defined as: myocardial infarctions, strokes, peripheral vascular disease and cardiovascular related death. Data were compared by non-parametric tests. Results 32 patients, 31 females, 12 diffuse SSc subsets were included. The control group included 30 age and sex matched patients without autoimmune diseases. Mean age of the group was 52 years±SD 9.7, mean disease duration was 8 years±SD 9. The prevalence of traditional risk factors was: 13% smokers, significant family history 38%, obesity 16%, dyslipidemia 32%, older age 13%, hypertension 16%. There were no significant differences from the control group. Major cardiovascular events were: 13% myocardial infarction, 9% peripheral vascular disease, 9% CV related deaths. Concerning CV risk indices of the 32 SSc patients, 4 (13%) were classified as having high CV risk according to QRiskII/SCORE/ ACC risk.. In SSc patients, we could not identify any correlation between the above mentioned risk indices and MACE, including death of cardiovascular causes, except for a slight correlation between the SCORE and cardiovascular related death (p=0.04). Conclusions In our study, the main prediction indices were not correlated with the 10 year risk for CV events in SSc patients suggesting that we need better risk prediction tools. Both traditional risk factors and endothelial dysfunction have been proposed to participate at the onset and progression of vascular disease in SSc. Special attention should be paid to correct the traditional risk factors in combination with specific treatment for SSc. References [1] Psarras A, Soulaidopoulos S, Garyfallos A, Kitas G, Dimitroulas T A critical view on cardiovascular risk in systemic sclerosis. Rheumatol Int2017;37(1):85–95. [2] Groseanu L, Bojinca V, Gudu T, Saulescu I, Predeteanu D, Balanescu A, et al. Low vitamin D status in systemic sclerosis and the impact on disease phenotype Eur J Rheumatol2016;3(2):50–55. Disclosure of Interest None declared

Aim. The study aims to assess the potential influences of rheumatoid arthritis (RA) and its specific disease measures on lean body composition phenotypes of female patients. Methods. The study was cross-sectionally designed to include Caucasian postmenopausal female RA patients and age-matched postmenopausal female controls. All the subjects gave written informed consent and the study was approved by the local ethics committee. Each subject underwent in the same day a clinical examination, laboratory tests, whole body dual X-ray absorptiometry (DXA) composition and physical activity estimation using a self-administered questionnaire. Correlations, differences and predictive power were analyzed with appropriate statistical tests. Results. The study included 107 RA patients and 104 controls. Compared to the normal subjects, who recorded higher levels of physical activity, the RA patients had significantly lower appendicular lean tissue absolute and relative indices and higher prevalence of sarcopenia. The whole body and appendicular lean tissue indices showed significant negative correlations with measures of disease severity (duration, inflammation, quality of life and radiographic progression), independent of age, levels of physical activity, body mass index and smoking. Conclusions. The measures of disease activity and severity independently predict lean tissue phenotypes in RA patients, behaving as risk factors for sarcopenia and rheumatoid cachexia. The diagnosis of RA in itself is a significant predictive factor of sarcopenia.

Guselkumab, a human monoclonal antibody that binds to the p19 subunit of interleukin 23, has been approved for the treatment of moderate-to-severe psoriasis. Psoriatic arthritis is a common comorbidity of psoriasis with an umet need for novel treatments. We assessed the efficacy and safety of guselkumab in patients with active psoriatic arthritis.We did a randomised, double-blind, placebo-controlled, phase 2a trial at 34 rheumatology and dermatology practices in Canada, Germany, Poland, Romania, Russia, Spain, and the USA. Eligible participants were aged 18 years or older with active psoriatic arthritis and plaque psoriasis affecting at least 3% of their body surface area, with three or more of 66 tender joints and three or more of 68 swollen joints, who had an inadequate response or intolerance to standard treatments. We randomly assigned patients (2:1) via a central interactive web-response system using computer-generated permuted blocks with a block size of six, stratified by previous anti-tumour necrosis factor-α use, to receive subcutaneous guselkumab 100 mg or placebo at week 0, week 4, and every 8 weeks thereafter for 24 weeks. Patients, investigators, and site staff were masked to treatment assignment until final database lock at week 56. At week 16, patients with less than 5% improvement in swollen and tender joint counts were eligible for early escape to ustekinumab. At week 24, the remaining placebo-treated patients crossed over to receive guselkumab 100 mg at weeks 24, 28, 36, and 44 and guselkumab-treated patients received a placebo injection at week 24, followed by guselkumab injections at weeks 28, 36, and 44. The primary endpoint was the proportion of patients with at least 20% improvement at week 24 in signs and symptoms of psoriatic arthritis according to American College of Rheumatology criteria (ACR20) in the modified intention-to-treat population (ie, all randomly assigned patients who received at least one dose of study treatment). Safety analyses included patients according to the study drug received. This study is registered with ClinicalTrials.gov, number NCT02319759.Between March 27, 2015, and Jan 17, 2017, we randomly assigned 149 patients to treatment: 100 to guselkumab and 49 to placebo. 17 (35%) of 49 patients in the placebo group and ten (10%) of 100 patients in the guselkumab group were eligible for early escape to ustekinumab at week 16. 29 (59%) of 49 patients in the placebo group crossed over and received guselkumab at week 24. Three (6%) of 49 patients in the placebo group, one (3%) of 29 patients who crossed over from placebo to guselkumab, and six (6%) of 100 patients in the guselkumab group discontinued study treatment before week 44. 58 (58%) of 100 patients in the guselkumab group and nine (18%) of 49 patients in the placebo group achieved an ACR20 response at week 24 (percentage difference 39·7% [95% CI 25·3-54·1]; p0·0001). Between week 0 and week 24, 36 (36%) of 100 guselkumab-treated patients and 16 (33%) of 49 placebo-treated patients had at least one adverse event. The most frequent adverse event was infection in both groups (16 [16%] of 100 patients in the guselkumab group vs ten [20%] of 49 patients in the placebo group). The prevalence of adverse events between week 0 and week 56 in guselkumab-treated patients (51 [40%] of 129) indicated no disproportional increase with longer guselkumab exposure. No deaths occurred.Guselkumab, a novel anti-interleukin 23p19 antibody, significantly improved signs and symptoms of active psoriatic arthritis and was well tolerated during 44 weeks of treatment. The results of this study support further development of guselkumab as a novel and comprehensive treatment in psoriatic arthritis.Janssen ResearchDevelopment.

Background Antiphospholipid syndrome (APS) is an autoimmune disease with wide clinical features and cumulative damage. The nervous system involvement is very broad and severe. Objectives The aim of this study is to analyze the impact of neurologic manifestations on Damage Index in Patients with APS (DIAPS). Methods All consecutive patients known with APS were included in our monocentric cohort. Data on medical history, clinical manifestations, aPL profile and medication were collected. DIAPS score was used to measure damage in each patient. Results Seventy six patients with APS were included: 11 patients with primary APS and 65 patients with secondary APS, with mean disease duration of 9.59±7.39years. Overall,35 patients (46.1%) had neurologic manifestations. Their mean disease duration was 9.2±5.76 years. Seven patients had primary APS and 28 patients had secondary APS. Six patients were on chronic oral anticoagulant therapy and low dose aspirin, 12 patients on oral anticoagulant alone and 15 patients on low dose aspirin. Transient ischemic attack was the first manifestation of APS in 4 patients (11.42%) at mean age of 29.5±10.96 years. Their mean DIAPS value was 7.75±4.19. Ischemic stroke was the first APS manifestation in 12 patients (34.28%) at mean age of 40.08±16.31years, with DIAPS mean value of 7.41±3.67. All of these patients have neurological sequelaes. The DIAPS value was higher in patients with neurologic manifestations (3±2.9 vs 5.71±3.62, p=0.001) and DIAPS value correlated significantly to neurologic manifestations (R=0.416, p Conclusions Neurologic manifestations in APS patients have a great impact on cumulative damage especially in patients presenting with ischemic stroke or transient ischemic attack as the first manifestation of APS. References M-C Amigo et al. Development and initial validation of a damage index (DIAPS) in patients with thrombotic antiphospholipid syndrome (APS). Lupus (2015) 24, 927–934. L.A. Martinez-Martinez et al. Damage Index in Patients with Thrombotic Antiphospholipid Syndrome: Retrospective Cohort Study. Ann Rheum Dis 2016;75:1065. Disclosure of Interest None declared

Background Antiphospholipid syndrome (APS) is an autoimmune disease defined as the presence of antiphospholipid antibodies (aPL), at least a clinical thrombotic event and is associated with an important risk of organ damage. The new index proposed, Damage Index in patients with Thrombotic Antiphospholipid Syndrome (DIAPS) may be an useful tool to estimate cumulative damage in patients with primary and secondary APS. It includes 38 clinical items expanded to show the complexity of clinical manifestations in APS patients. Objectives The aim of this study is to analyze the serological pattern as potential predictive factor for an increased DIAPS. Methods All consecutive patients known with APS according to the Sapporo and/or Sydney classification criteria were included in our monocentric cohort. Data on medical history, clinical manifestations, aPL profile and medication were collected. DIAPS score was used to measure damage in each patient. The relationship between aPL profile and DIAPS score was analysed. Results Seventy six patients with APS were included: 11 patients with primary APS, 65 patients with secondary APS. Their mean disease duration was 9.59±7.39years. The most frequent clinical manifestation from DIAPS was the peripheral vascular (deep vein thrombosis, intermittent claudication, tissue loss, vascular venous insufficiency) found in 61.8% of patients, followed by the neuropsychiatric manifestations (46.1%). The mean DIAPS score in our cohort was 4.25±3.51, not significantly different between patients with primary vs secondary APS (4.72 vs 4.16, p=0.629). Lupus anticoagulant (LA) was found in 25 patiens (32.9%), anti cardiolipin antibodies (aCL) in 49 patients (64.5%) and antibodies to β2-glycoprotein I (β2GPI) in 23 patients (30.3%). There were 36 patients known with a single positive aPL (47.4%), 27 patients (35.5%)with 2 positive aPL and only 2 patients with triple positivity. There were no significant differences regarding antibody profile between patients with primary and secondary APS. Higher values of DIAPS were seen in patients with β2GPI (p=0.042) and with positivity for 2 aPL (p=0.003). DIAPS value correlated to the presence of β 2GPI (p=0.042, R=0.233) and to positivity for two aPL (p=0.003, R=0.341). Conclusions Our study suggests that double positivity for aPL, especially the presence of β2GPI confers an increased value of DIAPS in patients with primary and secondary APS. References M-C Amigo et al. Development and initial validation of a damage index (DIAPS) in patients with thrombotic antiphospholipid syndrome (APS). Lupus (2015) 24, 927–934. LM Amezcua-Guerra. Improving definitions for an index of cumulative organ damage in patients with the antiphospholipid syndrome (DIAPS). Lupus (2016) 25, 671–672. Disclosure of Interest None declared

Background Systemic sclerosis (SSc) is a chronic multi-system autoimmune disease associated with disability and reduced quality of life. Objectives The purpose of this study was to assess health-related quality of life and disease perception in a group of SSc patients. Methods We performed a case-control study on 50 SSc patients from EUSTAR cohort 096. Socio-demographic data, disease characteristics and self-assessment questionnaires: Health assesment questionnaire (HAQ), EuroQol-5D (EQ5D) and the Brief Illness Perception Questionnaire were collected. Results The group included 41 females,31 limited SSc subsets. Medium HAQ value was 0.9 (0.6). Patients with higher Rodnan score (p=0.002), synovitis (p=0.02), late capillaroscopic pattern (p=0.02), muscle weakness (p=0.001), gastrointestinal involvement (p=0.01) and those on immunosuppressants (p=0.02) have a poor life quality. According to EQ-5D, the quality of life was related to specific organ involvement. 48% of the patients had some mobility problems, 6% were confined to bed; mobility was influenced by lung involvement (p=0.008), digital ulcers (p=0.03) and Medsger score (p=0.01). 48% of the patients had some self-care problems and 8% were not able to wash/dry themselves; self-care was influenced by the Rodnan score (p=0.02), diffuse subset (p=0.02), muscle weakness (p=0.03) and gastrointestinal involvement (p=0.021). 64% of the patients had some problems in performing usual activities and 16% were not able to perform them; the performance was influenced by disease subset (p=0.01), Medsger score (p=0.02), cardiac involvement (p=0.02) and the use of immunosuppressants (p=0.01). 52% of the patients had some and 38% had extreme pain/disconfort; 66% of the patients were moderately and 20% were extremly anxious/depressed. Both were related to digital ulcers (p=0.01, respectively p=0.045) The illness had a great impact on patients life 7.3 (2.5)/10. The main determinant was pulmonary fibrosis (p=0.04). The patients consider that their disease will continue for quite a long time 8.7 (2.6)/10. Most of the patiens do not feel to have a good control on their disease 6.3 (3.3)/10 and unfortunately they do not think that the treatment is very helpful 7.9 (2.7)/10. The intensity of the symptoms is quite severe 7.5 (2.7)/10,related to digital ulcers (p=0.04) and gastrointestinal involvement (p=0.02). Patients are very concerned about their disease 9.1 (2.3)/10, most of them being emotionally affected 7.6 (2.6). Conclusions This study confirms the presence and magnitude of impaired life quality in patients with SSc with impact on mobility, self-care, usual activities. The major determinants were the extend of skin involvement, musculoarticular, gastrointestinal involvement and digital ulcers. Often patients are anxious/depressed, had a high pain intensity and the perception of this illness is pessimistic. References Hudson M, Canadian Scleroderma Research Group Health-related quality of life in systemic sclerosis: a systematic review. Arthritis Rheum. 2009;61(8):1112–2. Frantz C et al. Impaired quality of life in systemic sclerosis and patient perception of the disease: A large international survey. Semin Arthritis Rheum. 2016;46(1):115–23. Disclosure of Interest None declared

Background Proper diagnosis of scleroderma is often long and difficult, since it is such a rare disease, and one which few doctors or patients are familiar with. Objectives To establish the interval between the symptoms9 onset of systemic sclerosis (SSc) and what type of investigations are performed until the patients reach the final diagnosis of a rheumatologist Methods This is a cross-sectional study that included randomly selected patients with a a diagnosis of SSc which were evaluated based on a questionnaire about symptoms at onset, specific consults and investigations. Descriptive statistics were used. Results The study group included 47 patients, of which only 5 were males and 17 from rural areas.The medium age was 53 (14.4) years. First symptom of onset was Raynaud phenomena 91.3% of the cases, followed by skin changes (56.5%), puffy fingers (52,2%), gastrointestinal and musculoarticular symptoms (23.9% each). The medium duration between the first symptom and a medical consult was 6 (63.5) months. The first medical consult was done by a internal medicine specialist -38.3%, by a rheumatologist-29.8%, a gastroenterologist-12.8%, a dermatologist-8.5%, a nephrologist and a pneumologist-4.2% and neurologist 2.1% The first suspected diagnosis was SSc in 14 cases, Raynaud syndrome in 8, connective tissue disease in 5, rheumatoid arthritis in 4, cancer, autoimmune hepatitis, idiopathic pulmonary fibrosis each in 2 cases and none in 10 cases. The medium number of consults until final diagnosis was 3.4 (1.7). The medium duration from the first symptom until the correct diagnosis was 39.2 (74) months. The first investigations recommended were blood tests in almost all of the patients (95.7%), but only a third of them included specific scleroderma autoantibodies. Capillaroscopy was performed as an initial diagnostic test in only 6 patients (12.8%). The mean interval from disease onset until the patient was referred to the first capillaroscopy was 13.5 (28.8) months, to specific autoantibodies was 40.17 (61.3) month, to echocardiography was 36.38 (54) months, to lung function tests and lung CT – 41.76 (65.8) months. There were no significant statistical differences between patients coming from rural environment and those coming from urban environment. The only significant statistical difference between diffuse and limited subset was the time the patient was referred to echocardiography (19.8 (47.6) months for the diffuse subset, 66.8 (94.6) months for the limited subset, p=0.04). The only statistical difference between males and females was related to the interval that capillaroscopy was performed (14 (20.5)months in females, 4.8 (5) months in males, p=0.02). Conclusions Scleroderma is a less well-known disease. This lack of awareness contributes to delayed diagnosis and delayed onset of therapy. Often such diagnostic uncertainty and frustration takes a huge toll on the psychological well-being of these patients, who describe their journey to diagnosis as being one of the most difficult part of their illness. One of our missions as rheumatologist is to increase recognition of this disorder. References https://www.sclero.org/scleroderma/diagnosis/difficult. Disclosure of Interest None declared

Background Psoriatic arthritis (PsA) is associated with important comorbidities: cardiovascular, gastro-intestinal, infectious, malignant, and psychiatric [1, 2]. However, they are less studied in PsA compared to other chronic inflammatory arthritis. Objectives The objective of this study was to calculate the prevalence of comorbidities and risk factors in a cohort of PsA patients. Methods This was an observational cross-sectional study, including consecutive, unselected adult patients, with a diagnosis of PsA according to their rheumatologist. Data collected: demographical, clinical (affected joints, current psoriasis, axial involvement, enthesitis, dactylitis), biological (acute phase reactants), and treatment related (nonsteroidal anti-inflammatory drugs, synthetic remissive drugs and biologics). Data on comorbidities and risk factors were collected according to the European League Against Rheumatism (EULAR) recommendations on reporting comorbidities in chronic inflammatory rheumatic diseases in daily practice [3]. Results In all, 129 PsA patients were included: 77 (59.7%) women, mean age ± standard deviation 53.5±11.8 years, disease duration 7±7.4 years; 53 (41.1%) had axial involvement, 33 (25.6%) dactylitis, 18 (14%) enthesitis, and 24 (18.6%) current moderate/severe psoriasis. Most of them had low or moderate disease activity and almost a quarter of them (32; 24.8%) were taking a biologic. The most prevalent comorbidities were: dyslipidaemia 103 patients (79.8%), hypertension 67 (51.9%), obesity 44 (34.1%), diabetes 21 (16.3%) and ischemic heart disease 15 (11.6%). Almost a third of patients (42, 32.6%) suffered a cardiovascular event after their PsA diagnosis, of which heart attack 2 patients, stroke 4, cardiac failure 4 and peripheral arterial disease one patient. Cardiovascular events correlated with smoking (r=0.893, p Regarding infectious comorbidities: 11 patients (8.5%) had a history of tuberculosis after being diagnosed with PsA, 7 (5.4%) chronic viral hepatitis, of which 4 with B virus and 3 with C virus, and 5 patients (3.9%) developed severe infections. Five patients (3.9%) were diagnosed with neoplasia, but no correlation was identified with any of the clinical, biological or treatment related included variables. Only 11 patients (8.5%) were diagnosed with depression, but the prevalence is probably underestimated, since not all patients were screened to this end. Conclusions PsA is associated with a high prevalence of comorbidities, especially cardiovascular diseases. This should be taken into consideration in the therapeutic and the global management of PsA patients. References Husni ME, Mease PJ. Managing comorbid disease in patients with psoriatic arthritis. Curr Rheumatol Rep 2010;12(4):281–7. Ogdie A, Yu Y, Haynes K, et al. Risk of major cardiovascular events in patients with psoriatic arthritis, psoriasis and rheumatoid arthritis: apopulation-based cohort study. Ann Rheum Dis 2015;74(2):326–32. Baillet A, Gossec L, Carmona L, et al. Points to consider for reporting, screening for and preventing selected comorbidities in chronic inflammatory rheumatic diseases in daily practice: a EULAR initiative. Ann Rheum Dis 2016;75(6):965–73. Disclosure of Interest None declared