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Background: The deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessively inherited disease resulting from loss-of-function mutations in ADA2, formerly named CECR1 (cat eye syndrome chromosome region, candidate 1) gene. Disease manifestations could be separated into three major phenotypes: inflammatory/vascular, immune dysregulatory and hematologic, however, most patients presented with significant overlap between these three phenotype groups. Due to its large heterogeneity, DADA2 is often mistaken with other connective tissue diseases. Over 60 DADA2-associated mutations have been identified so far, still we describe a new mutation with possible relation to severe hematological involvement Case presentation: We present a case of DADA2 deficiency with disease onset at 3 years old with fever, oligoarthritis, mouth sores and recurrent episodes of neutropenia misdiagnosed as Behcet disease, then systemic lupus erythematosus. At the age of 18 she developed gastrointestinal vasculitis complicated with multiple bowel resections, enterocutaneous fistulas and lower limb ischemia. Genetic testing reveals heterozygous mutations at positions 139 (exon 2) and 661_664 (exon 4) of the coding sequence of ADA2 (c.139G>A, c.661_664del), resulting respectively in amino acid substitution p.Gly47Arg and premature translational stop signal p.Ala221Glnfs*45 in ADA2. Thus, the patient was diagnosed with ADA2 deficiency. TNF blockers were started but the patient died due to septic complications related to severe, nonresponsive pancytopenia. Conclusions: Our case highlights the valuable diagnostic benefit of early specific genetic testing for patients with complex unclear diseases in which vasculopathy/vasculitis, dysregulated immune function and/or hematologic abnormalities may predominate.

Published by Elsevier Ltd., Background: Differences in the distribution of individual-level clinical risk factors across regions do not fully explain the observed global disparities in COVID-19 outcomes. We aimed to investigate the associations between environmental and societal factors and country-level variations in mortality attributed to COVID-19 among people with rheumatic disease globally. Methods: In this observational study, we derived individual-level data on adults (aged 18-99 years) with rheumatic disease and a confirmed status of their highest COVID-19 severity level from the COVID-19 Global Rheumatology Alliance (GRA) registry, collected between March 12, 2020, and Aug 27, 2021. Environmental and societal factors were obtained from publicly available sources. The primary endpoint was mortality attributed to COVID-19. We used a multivariable logistic regression to evaluate independent associations between environmental and societal factors and death, after controlling for individual-level risk factors. We used a series of nested mixed-effects models to establish whether environmental and societal factors sufficiently explained country-level variations in death. Findings: 14 044 patients from 23 countries were included in the analyses. 10 178 (72·5%) individuals were female and 3866 (27·5%) were male, with a mean age of 54·4 years (SD 15·6). Air pollution (odds ratio 1·10 per 10 μg/m3 [95% CI 1·01-1·17]; p=0·0105), proportion of the population aged 65 years or older (1·19 per 1% increase [1·10-1·30]; p, MAG is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (grant numbers K01 AR070585 and K24 AR074534 [JY]). KDW is supported by the Department of Veterans Affairs and the Rheumatology Research Foundation Scientist Development award. JAS is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (grant numbers K23 AR069688, R03 AR075886, L30 AR066953, P30 AR070253, and P30 AR072577), the Rheumatology Research Foundation (K Supplement Award and R Bridge Award), the Brigham Research Institute, and the R. Bruce and Joan M. Mickey Research Scholar Fund. NJP is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (T32-AR-007258). AD-G is supported by grants from the Centers for Disease Control and Prevention and the Rheumatology Research Foundation. RH was supported by the Justus-Liebig University Giessen Clinician Scientist Program in Biomedical Research to work on this registry. JY is supported by grants from the National Institutes of Health (K24 AR074534 and P30 AR070155).

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.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease of unknown etiology, presenting with variable clinical picture. Having a high heterogeneity and lacking pathognomonic features, very often the diagnosis poses a great challenge for the clinician. Several unusual clinical manifestations such as nasal septal perforation and digital gangrene can occur in LES patients. Case report. We report the case of a 42-year-old woman, known with SLE, hospitalized in our department for a clinical presentation consisting of a recent major epistaxis, physical asthenia and acral necrosis of the upper limbs. Physical examination revealed an afebrile patient, with a cushingoid facies, facial telangiectasias, and necrotic scars localized on the distal phalanges, bilaterally. A diagnostic nasal endoscopy showed a large septal perforation with the absence of the cartilaginous nasal septum. CT highlighted an extended defect at the level of the cartilaginous part of the nasal septum. Conclusion. Nasal septal perforation remains an underdiagnosed invalidating complication of lupus and treated and discovered early could have an important impact on the general health of an already burdened by disease patient.

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.

Background: Several studies described the cross-sectional characteristics of systemic sclerosis patients and coexisting primary biliary cholangitis, but longitudinal prognostic data are lacking. Aims: To describe the systemic sclerosis–primary biliary cholangitis phenotype, including baseline characteristics and outcomes. Methods: We performed a multicentre the European Scleroderma Trials and Research Group study of systemic sclerosis patients with primary biliary cholangitis or with primary biliary cholangitis–specific antibodies, matched with systemic sclerosis controls free from hepatobiliary involvement matched for disease duration and cutaneous subset. Data were recorded at baseline and at the last available visit. Results: A total of 261 patients were enrolled (115 primary biliary cholangitis–systemic sclerosis, 161 systemic sclerosis). At baseline, systemic sclerosis–primary biliary cholangitis patients had a higher prevalence of anti-centromere antibodies ( p = 0.0023) and a lower prevalence of complete absence of digital ulcers. The milder vascular involvement was confirmed at follow-up when crucial differences emerged in the percentage of patients experiencing digital ulcers; a significantly higher number of patients who never experienced digital ulcers were observed among primary biliary cholangitis–systemic sclerosis patients ( p = 0.0015). Moreover, a greater incidence of pulmonary arterial hypertension ( p Conclusion: Our data show that systemic sclerosis–primary biliary cholangitis exhibit a mild systemic sclerosis and primary biliary cholangitis phenotype with outcomes being in general favourable.

Summary Background Patients with primary systemic vasculitis or polymyalgia rheumatica might be at a high risk for poor COVID-19 outcomes due to the treatments used, the potential organ damage cause by primary systemic vasculitis, and the demographic factors associated with these conditions. We therefore aimed to investigate factors associated with COVID-19 outcomes in patients with primary systemic vasculitis or polymyalgia rheumatica. Methods In this retrospective cohort study, adult patients (aged ≥18 years) diagnosed with COVID-19 between March 12, 2020, and April 12, 2021, who had a history of primary systemic vasculitis (antineutrophil cytoplasmic antibody [ANCA]-associated vasculitis, giant cell arteritis, Behcet's syndrome, or other vasculitis) or polymyalgia rheumatica, and were reported to the COVID-19 Global Rheumatology Alliance registry were included. To assess COVID-19 outcomes in patients, we used an ordinal COVID-19 severity scale, defined as: (1) no hospitalisation; (2) hospitalisation without supplemental oxygen; (3) hospitalisation with any supplemental oxygen or ventilation; or (4) death. Multivariable ordinal logistic regression analyses were used to estimate odds ratios (ORs), adjusting for age, sex, time period, number of comorbidities, smoking status, obesity, glucocorticoid use, disease activity, region, and medication category. Analyses were also stratified by type of rheumatic disease. Findings Of 1202 eligible patients identified in the registry, 733 (61·0%) were women and 469 (39·0%) were men, and their mean age was 63·8 years (SD 17·1). A total of 374 (31·1%) patients had polymyalgia rheumatica, 353 (29·4%) had ANCA-associated vasculitis, 183 (15·2%) had giant cell arteritis, 112 (9·3%) had Behcet's syndrome, and 180 (15·0%) had other vasculitis. Of 1020 (84·9%) patients with outcome data, 512 (50·2%) were not hospitalised, 114 (11·2%) were hospitalised and did not receive supplemental oxygen, 239 (23·4%) were hospitalised and received ventilation or supplemental oxygen, and 155 (15·2%) died. A higher odds of poor COVID-19 outcomes were observed in patients who were older (per each additional decade of life OR 1·44 [95% CI 1·31–1·57]), were male compared with female (1·38 [1·05–1·80]), had more comorbidities (per each additional comorbidity 1·39 [1·23–1·58]), were taking 10 mg/day or more of prednisolone compared with none (2·14 [1·50–3·04]), or had moderate, or high or severe disease activity compared with those who had disease remission or low disease activity (2·12 [1·49–3·02]). Risk factors varied among different disease subtypes. Interpretation Among patients with primary systemic vasculitis and polymyalgia rheumatica, severe COVID-19 outcomes were associated with variable and largely unmodifiable risk factors, such as age, sex, and number of comorbidities, as well as treatments, including high-dose glucocorticoids. Our results could be used to inform mitigation strategies for patients with these diseases. Funding American College of Rheumatology and the European Alliance of Associations for Rheumatology.

Tumor necrosis factor (TNF) inhibitors are commonly utilized medications for the treatment of immune mediated conditions. Based on recent data from most registries there is no conclusive evidence for an increased risk of solid tumours or lymphoproliferative disease linked with biologic therapy, but on-going vigilance is required. We present a case of ankylosing spondylitis with extraarticular manifestations with a premalignant condition treated with TNF inhibitors, which developed a basal cell cancer, was switched on secukinumab and back again on antiTNF due to severe panuveitis.

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.

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).

B cell hyper-reactivity implied in the pathogenesis of Sjö gren’s syndrome (SS) justifies the therapy that involves B cell depletion. Ultrasound of salivary glands demonstrated alterations during disease progression. Objective. To evaluate changes in salivary gland parenchyma with ultrasound after rituximab treatment in patients’ with secondary SS (sSS). Methods. 7 patients evaluated at baseline (under treatment with rituximab) and after 6 months, underwent ultrasonography of major salivary gland (submandibular and parotid). Clinical data as dryness, pain and diseases activity evaluated with ESSDAI were registered. Salivary gland ultrasound (SGUS) was performed to asses echogenity (using a semiquantitative score from 0-4, with improvement defined as an at least 1 point decrease), size of gland and glandular borders. Results. Of 7 patients, 14% had clinically detectable bilateral parotid tumefaction at baseline. 6 patients (85.71%) showed ultrasonographic alterations at baseline. Parotid parenchyma echostructure improved in 42.85% of patients versus 14.28% in control group (p=0.05). At the submandibular glands, both submandibular glands showed changes in 28.57% of patients, while control group showed no changes. 42.85% patients showed parotid tumefaction which changed in 66.6% of patients at 6 months. Conclusion. Ultrasonography showed improvement in salivary gland echostructure in secondary Sjogren Syndrome with rituximab.

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.

We are presenting the case of a 48 years old female diagnosed at the age of 28 with diffuse cutaneous systemic sclerosis. During the course of disease, despite the vasodilatator treatments and immunosuppresion for visceral involvement, the patient developed multiple infected digital ulcers both in the upper limbs and in the lower limbs, which complicated with wet gangrene, requiring transmetatarsal amputations. At that time, a macrovascular disease or a prothrombotic condition were excluded. Patient also developed infected nondigital lower extremity ulcers, which slowly healed after Alprostadil treatment. The evolution of our patient‘s disease demonstrate that the management of scleroderma vasculopathy represent a challange and a multidisciplinary approach is needed.

We are presenting the case of a 36 years old male diagnosed at the age of 20 with reactive arthritis and at 27 years with ankylosing spondylitis. During the course of the disease, the patient followed treatment with nonsteroidal anti-inflammatory drugs, Sulfasalazine and biological therapy. The first two anti-TNF had a very good response. During Adalimumab therapy the patient conceived a child without any teratogenic effect on it. The effect of the two anti-TNF has been long-lasting, but the effectiveness has decreased on the other two. The future of this patient lies in blocking a new molecule in ankylosing spondylitis named IL-17, so we are expecting the disease response to the new biological introduced into ankylosing spondylitis, named Secukinumab.

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: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:Systemic sclerosis is associated with increased risk of cardiovascular disease (CVD) (1) and studies using MRI suggest that microvascular disease has an important role (2). Capillaroscopy is a non invasive and safe technique that assesses peripheral microvascular damage (3).Objectives:The objective of this study was to evaluate the predictive value of the capillaroscopy in relation to major adverse cardiovascular events (MACE).Methods:Retrospective study with three timepoints (at baseline, at 5 and 10 years) including patients with scleroderma from EUSTAR center 096. Data were collected from the registry and observation papers. We performed capillaroscopy to all patients at the time of inclusion in the EUSTAR registry (2004) and at baseline (2009). Also, CV risk scores were calculated at baseline and were reassessed at 5 and at 10 years using the SCORE calculator. Risk score was considered low if was between 0 and 2, moderate 3-9 and high >10. The relation between capillaroscopy and MACE was tested in using bivariate regression.Results:Of 22 patients, mean (standard deviation (SD)) age was 43.1 (11.5), all patients were females, mean (SD) disease duration was 5.4 (4.5). During the 10 years of follow up, 5 (22.7) patients had been lost of follow up and 8 (36.4) patients had died. Only 9 (40.9) patients completed the 10 year follow up.At the time of inclusion in the EUSTAR, one patient showed early scleroderma pattern at capilaroscopy, 15 had active pattern and 6 patients had late pattern. Capillaroscopic scoring showed 2 (9.1) patients with disarranged (50%) aspect, 4 (18.2) patients with local paucity, 10 (45.5) patients with enlarged loop bordering local paucity and 1 patient (4.5) with complete paucity.Capilaroscopy at baseline showed active pattern in 4 patients and late pattern in 18 patients. Thus, 13 (59.1) of patients had a progression of the disease at capilaroscopy before follow up.At baseline, 14 (63.6) patients had traditional CV risk factors. Cardiovascular risk scores found 21 (95.5) patients with a low risk score and 1 (4.5) patient with a moderate risk score. At 5 years 11 (0.5) patients had a low risk score and 1 (4.5) patient had moderate risk score and at 10 years, 5 (22.7) and 2 (9) patients had low and moderate risk scores, respectively. We found 6.2 MACE/100 patient-year and 5.5 deaths/100 patient-year.Capillaroscopy (p=0.684), disease progression on capillaroscopy (p=0.781), capillaroscopic scoring (p=0.92) and CV risk score (p=0.98) were not predictive factors of MACE.Conclusion:Patients with systemic sclerosis are at high risk of MACE and traditional CV risk scores underestimate this risk. Changes/progression on capilaroscopy is not predictive for MACE. However, this hypothesis needs to be tested on a bigger cohort.References:[1]Xintao Cen, SIning Feng, Shanshan Wei, Lu Wan, Ledong Sun, Systemic sclerosis and risk of cardiovascular disease: A PRISMA – compliant systemic review and meta-analysis of cohort studies, Medicine (Baltimore), 2020, 99(47)[2]N Galea, E Rosato, A Gigante, C Borrazzo, A Fiorelli et al, Early myocardial damage and microvascular dysfunction in asymptomativ patients with systemic sclerosis: A cardiovascular magnetic resonance study with cold pressor test, PLoS One 2020, 15 (12)[3]F Ingegnoli, R Gualtierotti, A systematic overview on the use and relevance of capillaroscopy in systemic sclerosis, Expert Rev CLin Ummunol, 2013, 9(11):1091-7Disclosure 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/Objective. Disease activity in spondyloarthritis (SpA) is evaluated through conventional scores such as BASDAI or ASDAS. The aim of this study was to determine disease activity in SpA patients under biological therapy with further assessment on the quality of each activity indicator and their clinical value together with their impact on NSAID intake. Materials and methods. Over nine months (January–August 2015) we enrolled 100 patients with SpA on antiTNF therapy (33% IFX; 35% ADL; 32% ETA). We collected demographic, clinical (BASDAI, ASDAS, PtGA) and laboratory data (ESR, CRP). Frequency of NSAID ingestion expressed as days per week was included in a questionnaire. Considering their biologic treatment, patients were divided into disease activity groups and sample characteristics were correlated and compared. Statistical analysis was performed with SPSS 20.0, using specific tests such as Pearson, Spearman or ANOVA. Results. Patients with active disease, stated by both activity scores had an increased NSAID intake. Out of 86% of patients with a BASDAI under 4, only 82.6% had an ASDAS-CRP lower than 1.3. BASDAI correlated to both ASDAS scores (r = 0.65 and 0.71, P

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