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PURPOSE To examine the impact of golimumab, on work productivity, activity limitation, and quality of life (QoL) in patients with axial spondyloarthritis (axSpA) and psoriatic arthritis (PsA). METHODS This real-world, multicenter, prospective study consecutively enrolled adult consented work-active patients with axSpA or PsA, newly initiated on golimumab as per the approved label. Prior receipt of > 1 prior biologic, or switching from another tumor-necrosis factor inhibitor due to primary non-response or safety reasons was not allowed. The Work Productivity and Activity Impairment-Specific Health Problem and the EuroQol 5-Dimensions (EQ-5D)-5-Level instruments were completed by the patients to assess the impact of golimumab on work productivity and activity impairment, and generic QoL, respectively. RESULTS Overall, 121 eligible patients (mean age: 45.4 years; median disease duration: 11.3 months), 51 diagnosed with PsA and 70 with axSpA, were enrolled by 19 rheumatologists. Over a 11.9-month median observation period

Microvascular dysfunction is the key element in the pathogenesis of systemic sclerosis (SSc), whereas the contribution of large and medium size vessel abnormalities is yet to be established. The aim of the present study is to assess the association between micro- and macrovascular function by utilizing a broad spectrum of assessments of vascular performance.We included consecutive, consenting SSc patients who underwent nailfold video capillaroscopy (NVC) for microcirculation evaluation. Peripheral and central systolic and diastolic blood pressure, carotid intima-media thickness (cIMT), aortic augmentation index (AIx) corrected for a heart rate of 75 beats per minute (AIx-75), and carotid-femoral pulse wave velocity (PWV) were also performed to assess macrovascular function. Cardiovascular risk disease (CVD) algorithms were also calculated and included in the analysis.A total of 81 patients (6 males) were studied with mean age 55.44 ± 13.40 years. Reduced capillary density was inversely correlated with arterial stiffness (Alx-75) and augmentation pressure (r = - 0.262, p = 0.018, and r = - 0.249, p = 0.025 respectively). Alx was significantly lower in the early compared to late pattern (28.24 ± 11.75 vs 35.63 ± 10.47, p = 0.036). A significant trend was found among NVC patterns with Alx-75 values being higher with the progression of microangiopathy towards the "late" group (26.36 ± 10.90 vs 30.81 ± 11.59 vs 35.21 ± 7.90, p = 0.027 for trend). Similarly, Framingham risk score and Atherosclerotic Cardiovascular Disease score were progressively higher across the worsening NVC patterns (4.10 ± 4.13 vs 2.99 ± 2.72 vs 6.36 ± 5.65, p = 0.023, and 6.99 ± 7.18 vs 5.63 ± 4.41 vs 12.09 ± 9.90, p = 0.019, respectively, for trends). Finally, QRISK3 (10-year cardiovascular disease risk) and ASCVD (Atherosclerotic Cardiovascular Disease) scores were inversely correlated with the number of capillaries (r = - 0.231, p = 0.048, and r = - 0.260, p = 0.038 respectively).These data suggest that CVD risk scores and macrovascular parameters are strongly correlated with microvasculopathy in patients with SSc. Key Points • Microangiopathy is the hallmark of SSc, but the relationship between subclinical atherosclerosis and small vessel disease remains unknown. • Arterial stiffening and CVD risk scores are positively associated with the degree of progression of peripheral microvasculopathy assessed with NVC. • The results of the study suggest an association between NVC abnormalities and higher CVD risk in SSc patients.

Objectives Predicting serious infections (SI) in patients with rheumatoid arthritis (RA) is crucial for the implementation of appropriate preventive measures. Here we aimed to identify risk factors for SI and to validate the RA Observation of Biologic Therapy (RABBIT) risk score in real-life settings. Methods A multi-centre, prospective, RA cohort study in Greece. Demographics, disease characteristics, treatments and comorbidities were documented at first evaluation and one year later. The incidence of SI was recorded and compared with the expected SI rate using the RABBIT risk score. Results A total of 1557 RA patients were included. During follow-up, 38 SI were recorded [incidence rate ratio (IRR): 2.3/100 patient-years]. Patients who developed SI had longer disease duration, higher HAQ at first evaluation and were more likely to have a history of previous SI, chronic lung disease, cardiovascular disease and chronic kidney disease. By multivariate analysis, longer disease duration (IRR: 1.05; 95% CI: 1.005, 1.1), history of previous SI (IRR: 4.15; 95% CI: 1.7, 10.1), diabetes (IRR: 2.55; 95% CI: 1.06, 6.14), chronic lung disease (IRR: 3.14; 95% CI: 1.35, 7.27) and daily prednisolone dose ≥10 mg (IRR: 4.77; 95% CI: 1.47, 15.5) were independent risk factors for SI. Using the RABBIT risk score in 1359 patients, the expected SI incidence rate was 1.71/100 patient-years, not different from the observed (1.91/100 patient-years; P = 0.97). Conclusion In this large real-life, prospective study of RA patients, the incidence of SI was 2.3/100 patient-years. Longer disease duration, history of previous SI, comorbidities and high glucocorticoid dose were independently associated with SI. The RABBIT score accurately predicted SI in our cohort.

Increased arterial stiffness is independently associated with renal function decline in patients with diabetes mellitus (DM). Whether DM has additional deleterious effects on central hemodynamics and arterial stiffness in chronic kidney disease (CKD) patients is yet unknown. This study aimed to compare ambulatory central BP, arterial stiffness parameters, and trajectories between patients with diabetic and non‐diabetic CKD. This study examined 48 diabetic and 48 non‐diabetic adult patients (>18 years) with CKD (eGFR

Nailfold videocapillaroscopy (NVC) changes in systemic sclerosis (SSc) are correlated with vascular complications, such as pulmonary arterial hypertension (PAH), supporting a potential link between peripheral and internal organ vasculopathy. The current stage of knowledge regarding NVC and PAH is discussed, focusing on the assessment of peripheral microangiopathy and a potential relationship with functional, echocardiographic, and haemodynamic markers of cardiac dysfunction. A comprehensive literature search was carried out to identify all studies focusing on NVC findings in patients with PAH, diagnosed with right heart catheterization. The majority of the studies examined NVC findings in patients with SSc-PAH, while three studies reported NVC abnormalities in patients with idiopathic PAH. Besides the pulmonary vasculature, a systemic component of microangiopathy seems to be involved in PAH. Well-designed prospective trials are warranted to validate NVC as a biomarker, with clinical implications in the diagnostic evaluation, risk stratification, and overall management of PAH in the daily clinical setting.

Systemic autoimmune inflammatory disorders confer a higher risk of cardiovascular (CV) disease leading to increased morbidity and mortality and reduced life expectancy compared to the general population. CV risk in systemic sclerosis (SSc) has not been studied extensively but surrogate markers of atherosclerosis namely carotid intima media thickness (cIMT) and pulse wave velocity (PWV) are impaired in some but not all studies in SSc patients. The aim of this study was to investigate the prevalence of subclinical atherosclerosis assessed by cIMT and PWV between two well-characterized SSc and Rheumatoid Arthritis (RA) cohorts. Consecutive SSc patients attending the Scleroderma Clinic were compared with RA patients recruited in the Dudley Rheumatoid Arthritis Co-morbidity Cohort (DRACCO), a prospective study examining CV burden in RA. Augmentation Index (Aix75) and cIMT were measured in all participants. Propensity score matching was utilised to select patients from the two cohorts with similar demographic characteristics, CV risk factors and inflammatory load. Unpaired analysis was performed using unpaired t test for continuous variables and χ2 test for dichotomous variables. Statistical analysis was repeated using paired t test for continuous normal variables and McNemar’s test for dichotomous variables. Fifty five age- and sex-matched SSc and RA patients were included in the analysis. No difference was demonstrated between SSc and RA subjects regarding cIMT (0.66 mm vs 0.63 mm, respectively) and Aix75% measurements (33.4 vs 31.7, respectively) neither in paired (p = 0.623 for cIMT and p = 0.204 for Aix%) nor in unpaired t test analysis (p = 0.137 for cIMT and p = 0.397 for AIx%). The results of this comparative study show that subclinical atherosclerosis is comparable between SSc and RA, a systemic disease with well-defined high atherosclerotic burden. Such findings underscore the importance of CV risk management in SSc in parallel with other disease-related manifestations.

Systemic sclerosis (SSc) is a systemic inflammatory, autoimmune disorder characterized by diffuse fibrosis of the skin and visceral organ involvement. Endothelial dysfunction and microvascular injury dominate the pathophysiology and clinical manifestations of the disease, while the impact of macrovascular atherosclerotic disease on cardiovascular (CVD) morbidity and mortality is yet to be established. In this article, we aim to review current knowledge about CVD as well as cardiac complications in SSc and discuss the potentially implicated pathogenetic mechanisms. Systemic inflammation has been identified as an important trigger and contributor for the development and progression of atherosclerosis, closely associated with high cardiovascular mortality in patients with autoimmune disorders, such as rheumatoid arthritis. A close interplay between traditional risk factors and factors related to the disease, including inflammation, endothelial injury, and immune-mediated cytotoxicity, sharing common pathogenetic features with microvasculopathy, may be responsible for large-vessel involvement and promotion of atherosclerosis in SSc. Cardiac complications, including heart failure due to impairment of coronary microcirculation and myocardial fibrosis, are listed among the primary cause of death in SSc. Evaluation of indirect surrogate markers of CVD, namely, arterial stiffness, carotid media thickness, and flow-mediated dilation, in small studies has provided inconsistent results regarding the association between SSc and atherosclerosis, highlighting the need for further research on this field. In this article, we aim to review current knowledge about large-vessel involvement and CVD in SSc and discuss the potentially implicated pathogenetic mechanisms. SSc conveys a higher risk for CVD associated with both vascular and fibrotic complications during the course of the disease. Increasing attention is given on the use of vasodilators, immunosuppressants, and more recently antifibrotic drugs that potentially improve myocardial function and reduce atherosclerotic disease burden.

Background Listeria monocytogenes is an opportunistic pathogen that causes severe infections of the Central Nervous System, such as meningitis or meningoencephalitis, and brain abscesses. Abscesses account for approximately 1-10% of CNS listerial infections and are observed in 1% of all listerial infections. Methods We describe a case of 70-year-old male patient who had several admissions in different hospitals over the last 8 weeks. Results He suffered from intermittent fever for over a month, recurrent episodes of headaches, disorientation and other neurological symptoms. His condition was misdiagnosed as giant cell arteritis and initially the patient was started on corticosteroids. MRI of the brain revealed the presence of multiple brain abscesses and the cerebrospinal fluid study confirmed the presence of Listeria Monocytogenes. The patient was started on ampicillin and he completed a 6 weeks' course of treatment. Conclusions This case emphasizes the need to include rare pathogens in the differential diagnosis when possible CNS infections are involved, as well as to show that in many cases some auto-immune diseases are overdiagnosed.

The therapeutic armamentarium in Systemic Lupus Erythematosus (SLE) is expanding with the introduction of novel biologic and small-molecule agents. Complementary to randomized controlled trials, registry-based studies are advantageous due to the inclusion of a wider range of patients from daily practice and the potential for long-term monitoring of the efficacy and safety of therapies. Moreover, data from registries can be used to identify disease phenotypes that best respond to biologic agents, and to correlate clinical response with parameters such as co-administered therapies and comorbidities. In this project, we will use the configuration of the Hellenic Registry of Biologic Therapies for inflammatory arthritides in order to design a dedicated SLE module with variables pertaining to global and organ-specific disease activity, severity, flares, organ damage/outcome, comorbidities and adverse events. The second stage will involve the pilot implementation of this platform for the multicentric registration of SLE patients who are treated with belimumab. The significance lies in the development of a structured registry that enables the assessment of the disease burden and the long-term efficacy and safety of existing and future biological agents in SLE. Piloting the registry can serve as a basis for establishing nationwide collaborative efforts.

Pulmonary arterial hypertension (PAH) represents one of the most devastating complications in connective tissue diseases (CTDs). The aim of this study was to investigate the presence of peripheral microangiopathy in patients with PAH associated with CTDs (CTD-PAH) by exploring nailfold videocapillaroscopic (NVC) changes and identify possible associations of NVC characteristics with markers of disease severity. Α cross-sectional study was performed in 18 CTD-PAH patients [13 PAH due to systemic sclerosis (SSc-PAH) and 5 with other types of CTD-PAH], 14 patients with SSc without PAH (SSc-non-PAH) and 20 healthy controls. NVC quantitative and qualitative parameters were evaluated using Optilia Digital Capillaroscope. To ensure inter-observer repeatability, capillaroscopic images were reviewed by two independent investigators. When compared to healthy controls, patients with CTD-PAH (77.8% women, mean age 65.9 years) presented reduced capillary density (6.5 ± 1.6 loops/mm vs. 9.7 ± 0.7 loops/mm, p

Background Although pulmonary vascular bed has been the main subject of research for many years in pulmonary hypertension (PH), interest has recently started to divert towards the possibility of a co-existing peripheral microangiopathy. The aim of the current study was to investigate the presence of nailfold video-capillaroscopic (NVC) structural changes in patients with precapillary PH and to identify possible associations of NVC measurements with markers of disease severity. Methods Α prospective case–control study was performed in 28 consecutive patients with precapillary PH [14 with idiopathic pulmonary arterial hypertension (IPAH) and 14 with chronic thromboembolic pulmonary hypertension (CTEPH)] and 30 healthy controls. NVC quantitative and qualitative parameters were evaluated using Optilia Digital Capillaroscope. To ensure inter-observer repeatability capillaroscopic images were reviewed by two independent investigators. For multiple comparisons among continuous variables, one-way ANOVA or the Kruskal–Wallis test were used. Differences between the groups were tested with post-hoc analysis with adjustment for multiple comparisons (Bonferroni test). Results Both IPAH (71.4% were women, mean age 53.1 ± 13.4 years) and CTEPH (64.3% women, mean age 60.9 ± 14.4 years) groups presented reduced capillary density compared to healthy controls (8.4 ± 1.2 loops/mm and 8.0 ± 1.2 loops/mm vs. 9.7 ± 0.81 loops/mm, p Conclusion The findings of the study reveal a degree of significant peripheral microvascular alterations in patients with IPAH and CTEPH, suggesting a generalized impairment of peripheral microvasculature in pulmonary vascular disease.

Introduction: Systemic sclerosis (SSc) is associated with a heightened cancer risk compared to the general population. Several pathways including immune system upregulation, cumulative inflammation, environmental factors, and genetic predisposition contribute to the development of both cancer and autoimmunity. Areas covered: This paper provides an overview of studies investigating the relationship between SSc and various types of cancer with a special focus on the identification of patients at higher risk for malignancy development. The demographic, serological, clinical, and disease-related characteristics of SSc individuals who are diagnosed with cancer over the course of their disease are discussed to provide a practical guidance for relevant screening strategies. Expert opinion: Several studies have identified subgroups of SSc patients at higher cancer risk based on the immunological profile (anti-RNAPol III positivity), diffuse disease type, and older age at SSc onset. Additionally, a close temporal association between SSc and cancer onset in certain antibody subsets raises the question as to whether more aggressive screening strategies should be considered. Currently, there are no published studies investigating the cost-effectiveness, efficacy, and safety of a targeted cancer-detection program. Screening procedures should at least follow recommendations for the general population with a special focus on patients at higher risk and specific cancer types.

Background: Data regarding the real-life predictors of low disease activity (LDA) in rheumatoid arthritis (RA) patients are limited. Our aim was to evaluate the rate and predictors of LDA and treatment patterns in RA. Methods: This was a multicenter, prospective, RA cohort study where patients were evaluated in two different time points approximately 12 months apart. Statistical analysis was performed in order to identify predictors of LDA while patterns of disease-modifying anti-rheumatic drug [DMARDs; conventional synthetic (csDMARD) or biologic (bDMARD)] and glucocorticoid (GC) use were also recorded. Results: The total number of patients included was 1317 (79% females, mean age: 62.9 years, mean disease duration: 10.3 years). After 1 year, 57% had achieved LDA (DAS28ESR3.2), 21% initiated (among csDMARDs users) and 22% switched (among bDMARDs users) their bDMARDs. Conclusion: In a real-life RA cohort, during 1 year of follow-up, 43% of patients do not reach treatment targets while only ~20% of those with active RA started or switched their bDMARDs. Male sex, younger age, lower HAQ, body mass index and co-morbidity index were independent factors associated with LDA while use of GCs or ⩾2 bDMARDs were negative predictors.

Eisenmenger syndrome (ES) comprises a severe phenotype of pulmonary arterial hypertension characterized by angiopathy of the lung circulation. The aim of the present study was to demonstrate the presence of systemic microvascular abnormalities in patients with ES using nailfold video-capillaroscopy (NVC) and to identify potential correlations of nailfold capillaroscopic characteristics with non-invasive markers of systemic organ function.Α cross-sectional NVC study was performed in 17 consecutive patients with ES and 17 healthy controls matched for age and sex. NVC quantitative (capillary density, capillary dimensions, haemorrhages, thrombi, shape abnormalities) and qualitative (normal, non-specific or scleroderma pattern) parameters were evaluated.Patients with ES [median age 40 (18-65) years, 11 women] presented reduced capillary density [8.8 (7.2-10.2) loops/mm vs. 9.9 (8.3-10.9) loops/mm, p = .004] and increased loop width [15.9 (10.3-21.7) μm vs. 12.3 (7.6-15.2) μm, p.001], while they had significantly more abnormal capillaries than healthy controls [2.5 (0.9-5.4) abnormal loops/mm vs. 1.0 (0.0-1.7) abnormal loops/mm, p.001]. NVC shape abnormalities in ES were positively correlated with NT-proBNP (r = 0.52, p = .03) and were negatively associated with estimated glomerular filtration rate (r = -0.60, p = .02). Additionally, capillary loop diameter was positively correlated with increased haemoglobin levels (r = 0.55, p = .03) and negatively correlated with reduced peripheral oxygen saturation (r = - 0.56, p = .02).This study supports the hypothesis of peripheral microvascular involvement in ES parallel to pulmonary microangiopathy detected by NVC. Further longitudinal studies are needed to confirm our preliminary results.

Background and Aims Blood pressure variability (BPV) is an important risk factor for cardiovascular events and mortality in patients with chronic kidney disease (CKD). Previous evidence suggests that BPV is gradually increasing across CKD stages. Whether type 2 diabetes mellitus (DM) is an additional risk factor for increased BPV has never been studied. The aim of this study is to examine in comparison BPV in diabetic and non-diabetic patients with CKD. Method We included 48 diabetic and 48 non-diabetic adult patients (>18 years) with CKD (eGFR: Results In total population, ambulatory systolic BP (SBP) levels were significantly higher in diabetics compared to non-diabetic counterparts in all studied periods. No significant differences were evidence for ambulatory diastolic BP (DBP) in total or across CKD stages. In total, 24-hour SBP SD (15.43±4.34 vs 13.38±3.35, p=0.011), wSD (14.41±4.11 vs 12.53±3.19, p=0.014) and ARV (10.94±2.75 vs 9.46±2.10, p=0.004) were higher in patients with DM compared to those without DM. In addition, 24hour DBP SD (11.04±2.39 vs 9.80±2.28, p=0.010), wSD (10.30±2.52vs 9.05±1.99, p=0.008), CV (14.77±3.05 vs 13.14±2.96, p=0.009) and ARV (8.23±2.10 vs 7.10±1.33, p=0.002) were again different between groups. Across CKD stages 2 and 3a, BPV indices were insignificantly higher in patients with DM. In CKD Stage 3b, 24-hour SBP-SD (16.30±4.52 vs 11.35±2.62, p=0.003), wSD (15.42±4.54 vs 10.77±2.30, p=0.004), ARV (12.46±3.19 vs 8.34±2.07, p=0.001) and 24-hour DBP-CV (14.84±3.63 vs 12.18±1.91, p=0.035) were higher in diabetic compared to non-diabetic patients. In contrast, no difference between groups existed in CKD Stage 4. Conclusion Patients with DM present increased BPV in CKD Stages 2, 3a and 3b (moderately impaired renal function). This difference is not apparent in patients with advanced CKD at Stage 4.

Background and Aims Αlterations in endothelial function and capillary circulation have been associated with increased cardiovascular events and overall mortality. Both diabetes mellitus (DM) and chronic kidney disease (CKD) have been associated with microcirculatory damage. Nailfold video-capillaroscory can provide a thorough assessment of capillary density and microcirculation changes. This is the first study examining in comparison microcirculatory function parameters in diabetic and non-diabetic patients with CKD. Method We included 48 diabetic and 48 non-diabetic adult patients (>18 years) with CKD (eGFR: Results Baseline demographic, anthropometric and laboratory characteristics were similar between patients with and without diabetes in total and in CKD stages. Overall, no significant differences at baseline capillary density were observed between groups; however diabetic patients presented significantly lower capillary density during reactive hyperemia (36.3±3.8 vs 38.3±4.3 capillaries/mm2, p=0.022) and at venous congestion (37.8±4.0 vs 39.8±4.2 capillaries/mm2, p=0.015). When stratified according to CKD stages, the between-group differences in parameters of interest were not significant in stages 2, 3a and 4. In stage 3b, capillary density was significantly lower in diabetic compared to non-diabetic subjects at baseline (31.1±2.8 vs 33.4±3.4 capillaries/mm2, p=0.044), during postocclusive hyperemia (36.8±2.7 vs 40.0±4.3 capillaries/mm2, p=0.037) and venous congestion (38.3±2.8 vs 41.5±3.5 capillaries/mm2, p=0.022). Conclusion Capillary density during postocclusive reactive hyperemia and after venous congestion is lower in diabetic compared to non-diabetic CKD patients, a finding indicative that diabetes is an additional factor contributing to microcirculatory functional impairment in CKD. These differences are more prominent in CKD stage 3b, and less prominent in earlier and later stages.

Cardiovascular disease is the main cause of mortality in chronic kidney disease (CKD). Endothelial dysfunction and capillary rarefaction are established cardiovascular risk factors. Nailfold video capillaroscopy provides a thorough assessment of capillary density and functional reserve. This study aimed to examine possible differences in structural and functional capillary density in CKD stages 2–4 with nailfold video capillaroscopy. Ninety-six CKD patients, divided into four equally sized groups according to CKD stage (2, 3a, 3b, 4), underwent nailfold video capillaroscopy, during which capillary density was measured at baseline, after 4-min arterial occlusion and after 2-min venous occlusion. Arterial stiffness and wave parameters were measured with applanation tonometry and common carotid intima-media thickness (ccIMT) with ultrasound. Baseline capillary density showed a progressive reduction with advancing CKD stages (stage 2: 32.6 ± 2.8, stage 3a: 31.2 ± 3.8, stage 3b: 32.5 ± 3.3, stage 4: 28.5 ± 3.1, p = 0.011). Similar reductions were observed during postocclusive hyperemia (39.4 ± 3.0, 37.6 ± 4.2, 38.4 ± 3.8, and 33.8 ± 3.3, respectively; p = 0.021) and after venous congestion (41.1 ± 3.1, 39.0 ± 4.4, 39.9 ± 3.5, and 35.2 ± 3.4; p = 0.032). Office PWV and ccIMT showed nonsignificant increasing trends with advancing CKD. In multivariate analysis, eGFR showed a positive association (per ml/min increase; β: 0.053, 95% CI: 0.004–0.101), whereas diabetes (β: −1.706, 95% CI: −3.176 to −0.236) and parathyroid hormone (PTH) (per pg/ml increase; β: −0.022, 95% CI: −0.036 to −0.008) had negative associations with postocclusive capillary density. Both structural and functional capillary density progressively decrease with advancing CKD stages. Apart from reduced eGFR, diabetes and increased PTH levels are independently associated with this reduction. This capillary rarefaction may largely contribute to the increased cardiovascular risk of CKD patients.

Objectives Evidence on comorbidity prevalence in antiphospholipid syndrome (APS) and its difference from high comorbidity burden rheumatic diseases is limited. Herein, we compare multiple comorbidities between APS and RA. Methods A total of 326 patients from the Greek APS registry [237 women, mean age 48.7 (13.4) years, 161 primary APS (PAPS), 165 SLE-APS] were age/sex matched (1:2 ratio) with 652 patients from a Greek multicentre RA cohort of 3115 patients. Prevalence of cardiovascular (CV) risk factors, stroke, coronary artery disease (CAD), osteoporosis, diabetes mellitus (DM), chronic obstructive pulmonary disease (COPD), depression and neoplasms were compared between APS and RA patients using multivariate regression analysis. Results Ηyperlipidemia and obesity (ΒΜΙ ≥ 30 kg/m2) were comparable while hypertension, smoking, stroke and CAD were more prevalent in APS compared with RA patients. Osteoporosis and depression were more frequent in APS, while DM, COPD and neoplasms did not differ between the two groups. Comparison of APS subgroups to 1:2 matched RA patients revealed that smoking and stroke were more prevalent in both PAPS and SLE-APS vs RA. Hypertension, CAD and osteoporosis were more frequent only in SLE-APS vs RA, whereas DM was less prevalent in PAPS vs RA. Hyperlipidaemia was independently associated with CV events (combined stroke and CAD) in PAPS and SLE-APS, while CS duration was associated with osteoporosis in SLE-APS. Conclusion Comorbidity burden in APS (PAPS and SLE-APS) is comparable or higher than that in RA, entailing a high level of diligence for CV risk prevention, awareness for depression and CS exposure minimization.

To describe the profile of Enthesitis Related Arthritis’ (ERA) patients, in the era of biologic DMARDs (bDMARDs). This retrospective cohort study included patients with ERA monitored on a 3-month schedule for at least 1 year. Their metric assessment included the disease status and damage by applying the contemporary tools clinical-Juvenile Arthritis Disease Activity Score (c-JADAS), Juvenile Spondyloarthritis Disease Activity Index (JSpADA), clinical remission (CR) on/off medication and Juvenile Arthritis Damage Index (JADI). 43 patients (males 26) were enrolled, with a mean disease onset of 10.75 years. Median lag time from diagnosis to bDMARDs was 8.5 months. Patients with sacroiliitis received earlier bDMARDs (hazard ratio, HR 3.26). 36/43 patients achieved CR on medication (median time 11 months), which was correlated with compliance (HR: 3.62). The percentage of CR in patients with or without sacroiliitis was 35% and 63% respectively (p = 0.02). Twenty patients (47%) experienced a flare following CR (75%). The median flare-free survival following CR on/off medication was 42 and 34 months, respectively. At the last evaluation, both median baseline cJADAS and JSpADA dropped to 0, 13/43 patients had a persistent disease activity, while 17/43 and 13/43 patients were in CR on/off medication, respectively. The median patient percentage of CR was 54% and no patient had a JADI > 0. Increased lag time to bDMARDs was associated with increased CR (Odds ratio: 1.48). Early administration of bDMARDs and compliance improved long-term outcome of ERA. Sacroiliitis was a negative prognostic factor with an increased need for bDMARDs and diminished rates of CR.

Objective Endothelial dysfunction has been associated with increased cardiovascular events and overall mortality. Microvascular damage is prevalent both in diabetes mellitus (DM) and chronic kidney disease (CKD). Our aim was to compare microcirculatory function parameters in diabetic and non-diabetic CKD patients via nailfold video-capillaroscopy. Methods We included 48 diabetic and 48 non-diabetic adult CKD patients. All participants underwent nailfold video-capillaroscopy, during which capillary density was measured at normal conditions (baseline), after a 4-minute arterial occlusion (postocclusive reactive hyperemia), and at the end of a 2-minute venous occlusion (congestion phase). Results Diabetic patients presented significantly lower capillary density during reactive hyperemia (36.3 ± 3.8 vs 38.3 ± 4.3 capillaries/mm2 , P = .022) and at venous congestion (37.8 ± 4.0 vs 39.8 ± 4.2 capillaries/mm2 , P = .015). When stratified according to CKD stages, only in stage 3b capillary density was significantly lower in diabetic compared to non-diabetic subjects at baseline, during postocclusive hyperemia (36.8 ± 2.7 vs 40.0 ± 4.3 capillaries/mm2 , P = .037) and venous congestion (38.3 ± 2.8 vs 41.5 ± 3.5 capillaries/mm2 , P = .022). Conclusions Capillary density during postocclusive hyperemia and after venous congestion is lower in diabetic compared to non-diabetic CKD patients, a finding indicative that diabetes is an additional factor contributing to microcirculatory structural and functional impairment in CKD. These differences are more prominent in CKD stage 3b.

Inflammasomes are large intracellular complexes that induce inflammation in response to exogenous and endogenous damage signals. They regulate production and release of the proinflammatory cytokines IL-1β and IL-18, playing a defensive role against infections. Inflammasomes have also been involved in the pathogenesis of a wide range of autoinflammatory conditions that are caused by dysregulation of the IL-1 pathway, such as cryopyrinopathies and hereditary periodic fever syndromes. On top of that, research in recent years suggests that defects in inflammasome regulation and signaling associate with a number of autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and others. In this review, we describe the inflammasome and mechanisms that trigger it, provide a brief review of autoinflammatory disorders and discuss the current understanding and emerging data from experimental and clinical studies for the role of the innate immune system and inflammasomes in the biology and pathogenesis of systemic autoimmune diseases.

Aim of the study To evaluate the current disease characteristics, treatment and comorbidities of rheumatoid arthritis (RA) in Greece. Methods Multicenter, cross-sectional study with a 9-month recruitment period between 2015 and 2016. Demographics, disease characteristics, treatment and comorbidities were collected via a web-based platform. Results 2.491 RA patients were recruited: 96% from tertiary referral centers, 79% were females with a mean age of 63.1 years and disease duration of 9.9 years. Fifty-two percent were rheumatoid factor and/or anti-CCP positive, while 41% had erosive disease. Regarding treatment, 82% were on conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs), 42% on biologic DMARDs (TNFi: 22%, non-TNFi: 20%) and 40% on corticosteroids (mean daily dose: 5.2 mg). Despite therapy, 36% of patients had moderate and 12% high disease activity. The most frequent comorbidities were hypertension (42%), hyperlipidemia (33%), osteoporosis (29%), diabetes mellitus (15%) and depression (12%). Latent tuberculosis infection (positive tuberculin skin test or interferon gamma release assay) was diagnosed in 13 and 15.3% of patients, respectively. Regarding chronic viral infections, 6.2% had history of herpes zoster while 2% and 0.7% had chronic hepatitis B and C virus infection, respectively. A history of serious infection was documented in 9.6%. Only 36% and 52% of the participants had ever been vaccinated against pneumococcus and influenza virus, respectively. Conclusion This is one of the largest epidemiologic studies providing valuable data regarding the current RA characteristics in Greece. Half of patients were seropositive but despite therapy, half displayed residual disease activity, while preventive vaccination was limited.

Objectives To describe the disease characteristics, continuous course and long-term outcome and to evaluate predictors of outcome in JIA in Greece. Methods We performed a retrospective cohort analysis of 17 years' prospective data on JIA. Outcome assessment included radiographic (modified Sharp-van der Heidje score), articular and extra-articular damage (Juvenile Arthritis Damage Index), functional ability (HAQ Disability Index), and the cumulative percentage time spent in a state of active disease and also in clinical remission off medication (CR) (according to Wallace's criteria). Results One hundred and two (72 females) patients under regular follow-up were enrolled. The disease age of onset [mean (SD)] was 7.7 (4) years, the interval from onset to last visit was 17.2 (6.7) years and the patients' current age was 25 (5.9) years. At the last follow-up visit, 53 patients (52%) had disease activity, while 23.5% were in CR. The cumulative percentage time spent in a state of active disease and CR over the disease course was 52.6 and 17.8%, respectively. Polyarticular subtype of onset and longer disease activity during the first 5 years were independent predictors of worse outcome. Additional telephone-based interviews of 205 former JIA patients who had been lost to follow-up as adults were performed to extend the interpretation of our findings to a broader JIA population. Almost half (47.6%) of the total cohort of 307 patients were found to be in CR at the final evaluation and 69.7% had no disability. Conclusion The available data indicate that JIA as a whole is a heterogeneous disease with significant variability in course and long-term outcome.

Background Our previous publication on Juvenile Idiopathic Arthritis (JIA) revealed that half of the pts had flares in adulthood, despite previous administration of several anti-TNFs. Objectives a. To describe the long-term outcome of adults with JIA or non-infectious uveitis (NIU), exposed to Adalimumab (ADA) as the single or last biologic during the transition period. b. To evaluate our model of transition in a subgroup of refractory to conventional DMARDs JIA or NIU pts, in the 1st Greek Transition Clinic for Pediatric Rheumatic Diseases. Methods Retrospective cohort analysis of Greek adults with JIA or NIU between 2004 and 2018. The JIA activity state was assessed by 2 quantitative tools: the Juvenile Arthritis Disease Activity Score-10 (JADAS-10, Clinical Remission [CR], Low, Moderate and High Disease Activity [LDA, MDA, HDA], respectively) and Wallace criteria (CR on/off ADA). Baseline was defined as the 1st ADA dose Results 28 pts were enrolled: 24 JIA pts (female 18), aged 20.6±3.1 yrs, for a f/u of 6.5±3 years (Group-A) and 4 female pts with active uveitis (2 with JIA-associated and 2 with NIU, mean aged: 18.9 yrs, f/u: 2.3 yrs) (Group-B). The age at baseline was 14.1±3.2 yrs in Group-A and 16.7 yrs in Group-B. The lag time from JIA onset to baseline was 6.3±3.8 yrs and the JIA subtypes were: 37.5% polyarthritis RF neg, 33.3% extended oligoarthritis, 20.8% enthesitis-related arthritis, 4.2% persistent oligoarthritis, 4.2% psoriatic arthritis. 11/24 (45.8%) pts (Group-A) and 3/4 pts (Group-B) were ANA positive, 5/24 JIA-pts had a history of uveitis (20.8%). Naive to anti-TNF drugs were 18 pts (75%) of Group-A and 4/4 from Group-B. The baseline JADAS-10 was 13.9±5.9. The ADA yr-administration was 5.3±2.3 in Group-A and 2 in Group-B. Compliance to ADA had 17/24 (70.8%) pts in Group-A and all from Group-B. Regarding safety, 4/28 patients (14.3%) experienced Events of Special Interest (1/33.6 patient-years), herpes zoster (n=1), anorexia nervosa (n=1), breast fibroadenoma (n=1) and uveitis (n=1). Among the Group-A pts: 4/24 (16.7%) discontinued ADA due to inefficacy and 1/24 due to pregnancy planning. 6/24 pts (25%) achieved CR and discontinued ADA after 5.3±1.6 yrs. CR off ADA lasted 15.5±9.8 mo. The rest 13/24 continued ADA at the last f/u. In Group-B, 3/4 were still on ADA and 1 pt discontinued ADA due to NIU remission after 4.2 yrs and sustained CR 16 mo off ADA. The median% of CR remission on ADA total administration period was 75% in Group-A and 100% in Group-B. Among the ongoing receivers: a) the JIA activity state at the last assessment was: CR 84.6%, LDA 7.7% MDA 7.7% (median JADAS: 0, Group-A), b) 100% were in remission in Group-B. Conclusion 86% of adult pts with JIA or NIU tolerate ADA well. Pts exposed to ADA achieved CR on/off medication in 75% and 25%, respectively. References [1] Dimopoulou D, et al. Rheumatology (Oxford) 2017 *: Despoina Dimopoulou and Maria Trachana contributed equally Disclosure of Interests None declared

Background Identification of the risk factors for the development of serious infections in patients with rheumatoid arthritis (RA) is critical for designing preventive measures and early treatment. Objectives To identify risk factors for serious infections and to validate the RABBIT risk score in RA patients in daily clinical practice. Methods Multicenter, prospective study of RA patients in Greece. Demographics, disease characteristics, treatments and co-morbidities were prospectively collected via a web-based platform at baseline and one year later. The observed incidence of serious infections was compared to the one estimated by the RABBIT score. Results 1.549 RA patients were included (women: 78%, mean age: 63 years, mean disease duration: 10.4 years, RF and/or anti-CCP positive: 54%, mean DAS28: 3.4, mean HAQ: 0.5, bDMARD use: 46%, corticosteroid use: 36%). During follow-up, 38 serious infections were recorded (incidence: 2.3/100 patient-years). Patients who developed a serious infection had longer disease duration (14.2 vs. 10.3 years, p=0.02), higher HAQ at baseline (0.85 vs. 0.5, p=0.006), a history of previous serious infection (26% vs. 10%, p=0.002) and were more likely to have chronic lung disease (23% vs. 9%, p=0.008) or cardiovascular disease (23% vs. 11%, p=0.04). Disease duration (OR: 1.04, CI: 1.01-1.08, p=0.025), history of previous infection (OR: 3.3, CI: 1.3-8.1, p=0.01) and chronic lung disease (OR: 3.03, CI: 1.17-7.85, p=0.023) remained statistical significant in multivariate logistic regression analysis. bDMARD use was not associated with increased risk in uni-or multi-variate analysis. Data for RABBIT score calculation were available in 1.349 patients. Estimated incidence per 100 patient-years was divided in Q1-Q4 quartiles (25-50-75th percentiles: 0.94-1.35-2.15). Estimated and observed incidence rates were in Q1: 0.8 and 0.54, Q2: 1.14 and 0.79, Q3: 1.76 and 1.84 and Q4: 3.7 and 5.6, respectively (Hosmer Lemeshaw test, p=0.9). Conclusion In this large, real life, prospective study of RA patients, the incidence of serious infections was 2.3/100 patient-years. Longer disease duration, history of previous serious infections and chronic lung disease were independently associated with the development of serious infections. RABBIT score predicted rather accurately the risk for serious infections in our cohort. References [1] Zink et al, Ann Rheum Dis. 2014 Sep; 73(9): 1673–1676. Acknowledgement Supported by grants from the Greek Rheumatology Society and Professional Association of Rheumatologists. Disclosure of Interests Konstantinos Thomas: None declared, Argyro Lazarini: None declared, Evripidis Kaltsonoudis: None declared, Alexandros Drosos: None declared, Ioannis Papalopoulos: None declared, Prodromos Sidiropoulos: None declared, Panagiota Tsatsani: None declared, Sousana Gazi: None declared, Lina Pantazi: None declared, Kyriaki Boki: None declared, Pelagia Katsimbri: None declared, Dimitrios Boumpas: None declared, Kalliopi Fragkiadaki: None declared, Maria Tektonidou: None declared, Petros Sfikakis: None declared, Konstantina Karagianni: None declared, Lazaros Sakkas: None declared, Gerasimos Evangelatos: None declared, Alexios Iliopoulos: None declared, Eleftheria Grika: None declared, PANAYIOTIS VLACHOYIANNOPOULOS: None declared, Theodoros Dimitroulas: None declared, Alexandros Garyfallos: None declared, Konstantinos Melissaropoulos: None declared, Panagiotis Georgiou: None declared, Maria Areti: None declared, Constantinos Georganas: None declared, Periklis Vounotrypidis: None declared, Georgios Georgiopoulos: None declared, George D Kitas Speakers bureau: GDK has received honoraria for lectures, participation in advisory boards and/or hospitality by Roche, Abbvie, Pfizer, Novartis, UCB, BMS, GSK and received grant support from Lilly., Dimitrios Vassilopoulos: None declared

Background: Giant cell arteritis (GCA) usually requires long-term therapy with corticosteroids and/or Disease Modifying Anti-rheumatic Drugs (DMARDs) but the exact factors that are associated with drug discontinuation in these patients have not been well defined. Objectives: To evaluate the baseline or on-treatment factors that influence drug survival in GCA. Methods: Data were derived from an ongoing, multicenter, prospective cohort study of patients with GCA. During the 1st phase of the study, data regarding demographic and clinical characteristics at baseline, type of treatment, adverse events of therapy and co-morbidities were retrospectively collected and analyzed. Predictors of drug discontinuation were examined by univariate and multivariate logistic regression analyses. Results: One hundred and seventy seven patients were included in the study; 70% (n=120) were women with a mean age at diagnosis of 74 ± 8.4 years. Diagnosis was established by temporal artery biopsy in 127 patients (72%), ultrasound of temporal arteries in 37 patients (21%) and large vessel imaging in 10 patients (6%). At diagnosis, the median ESR and CPR were 103 mm/h and 63 mg/L, respectively. All patients were treated initially with pos corticosteroids (median daily starting dose: 41 mg), while 12 (7%) of patients received pulse steroids. At the 1st patient evaluation (median follow-up from diagnosis: 3 years), the median daily steroid dose was 5 mg, while in 34% (n= 62) and 8% (n= 14) of patients a synthetic or biologic DMARD had been added, respectively. During that period, 24% (n=43) of patients had discontinued corticosteroids and 18% (n=32) all treatments. By univariate analysis, DMARD use was associated with a higher possibility for corticosteroid discontinuation (OR=2.3, p=0.017) but by multivariate logistic regression analysis only female sex (OR=0.49, p=0.07) and age at diagnosis (OR=0.94, p=0.01) were associated with a decreased risk for corticosteroid discontinuation. Similar results were found for discontinuation of all drugs [female sex (OR=0.4, p=0.05) and age at diagnosis (OR=0.9, p=0.046)]. Conclusion: In this large GCA cohort, only one out of four patients managed to discontinue corticosteroids and 20% all treatments ∼3 years after diagnosis. Multivariate analysis revealed only female sex and age at diagnosis as independent factors for treatment discontinuation. Acknowledgement: Supported by grants from the Greek Rheumatology Society and Professional Association of Rheumatologists. Disclosure of Interests: None declared

Systemic sclerosis (SSc) is a chronic, systemic disease characterized by fibrosis of the skin and internal organs, vasculopathy, and auto-immune activation. On the top of severe organ involvement such as interstitial lung and myocardial fibrosis, pulmonary hypertension, and renal crisis, individuals diagnosed with SSc may suffer from a number of comorbidities. This is a narrative review according to published recommendations and we searched the online databases MEDLINE and EMBASE using as key words the following terms: systemic sclerosis, scleroderma, myocardial fibrosis in combination with micro- and macro-vascular disease, cardiac involvement, atherosclerosis, cardiovascular disease and coronary arteries, infections, cancer, depression, osteoporosis, and dyslipidemia. Although data are usually inconclusive it appears that comorbidities with significant impact on life expectancy, namely cardiovascular disease, infections, and cancer as well as phycological disorders affecting emotional and mental health are highly prevalent in SSc population. Thereafter, the aim of this review is to summarize the occurrence and the clinical significance of such comorbidities in SSc population and to discuss how rheumatologists can incorporate the management of these conditions in daily clinical practice.

Spondyloarthropathies (SpA) include many different forms of inflammatory arthritis and can affect the spine (axial SpA) and/or peripheral joints (peripheral SpA) with Ankylosing spondylitis (AS) being the prototype of the former. Extra-articular manifestations, like uveitis, psoriasis and inflammatory bowel disease (IBD) are frequently observed in the setting of SpA and are, in fact, part of the SpA classification criteria. Bowel involvement seems to be the most common of these manifestations. Clinically evident IBD is observed in 6%-14% of AS patients, which is significantly more frequent compared to the general population. Besides, it seems that silent microscopic gut inflammation, is evident in around 60% in AS patients. Interestingly, occurrence of IBD has been associated with AS disease activity. For peripheral SpA, two different forms have been proposed with diverse characteristics. Of note, SpA (axial or peripheral) is more commonly observed in Crohn's disease than in ulcerative colitis. The common pathogenetic mechanisms that explain the link between IBD and SpA are still ill-defined. The role of dysregulated microbiome along with migration of T lymphocytes and other cells from gut to the joint ("gut-joint" axis) has been recognized, in the context of a genetic background including associations with alleles inside or outside the human leukocyte antigen system. Various therapeutic modalities are available with monoclonal antibodies against tumour necrosis factor, interleukin-23 and interleukin-17, being the most effective. Both gastroenterologists and rheumatologists should be alert to identify the co-existence of these conditions and ideally follow-up these patients in combined clinics.

Background Comorbidities are common in chronic systemic connective tissue diseases and are associated with adverse outcomes, increased morbidity and mortality. Although the prevalence of comorbidities has been well-studied in isolated diseases, comparative studies between different autoimmune diseases are limited. In this study, we compared the prevalence of common comorbidities between patients with systemic sclerosis (SSc) and patients with rheumatoid arthritis (RA). Methods Between 2016 and 2017, 408 consecutive patients with SSc, aged 59 ± 13 years (87% women), were matched 1:1 for age and gender with 408 patients with RA; mean disease duration was 10 ± 8 and 9 ± 8 years, respectively. Rates of cardiovascular risk factors, coronary artery disease, stroke, chronic obstructive pulmonary disease (COPD), osteoporosis, neoplasms and depression were compared between the two cohorts. Results The prevalence of dyslipidemia (18.4% vs 30.1%, p = 0.001) and diabetes mellitus (5.6% vs 11.8%, p = 0.007) and body mass index (p = 0.001) were lower in SSc compared to RA, while there was no difference in arterial hypertension or smoking. While there was a trend for lower prevalence of ischemic stroke in SSc than in RA (1.1% vs 3.2%, p = 0.085), coronary artery disease was comparable (2.7% vs 3.7%). No differences were found between patients with SSc and patients with RA in the prevalence of COPD (5.2% vs 3.7%), osteoporosis (24% vs 22%) or neoplasms overall (1.1% vs 1.7%); however lung cancer was the most prevalent cancer in SSc (7/17, 41%), whereas hematologic malignancies (7/19, 36%) and breast cancer (7/19, 36%) predominated in RA. Depression was more prevalent in SSc (22% vs 12%, p = 0.001), especially in diffuse SSc. Conclusions Despite the prevalence of dyslipidemia and diabetes mellitus in SSc being almost half that in RA, the cardiovascular comorbidity burden appears to be similar in both. The overall prevalence of neoplasms is no higher in SSc than in RA, but SSc has a more negative impact on quality of life, as clearly, more SSc patients develop depression compared to patients with RA.

Double Negative (DN) B cells constitute a B cell population that lacks expression of immunoglobulin D and CD27 memory marker. These cells expand in elderly healthy individuals, but also accumulate prematurely in autoimmune and infectious diseases. COVID-19 is a pandemic infectious disease caused by SARS-CoV-2, a coronavirus that was first observed in Wuhan, China in December 2019. In its more severe cases, COVID-19 causes severe pneumonia and acute respiratory syndrome with high morbidity and mortality. Recent studies have revealed that the extrafollicular DN2 B cell subset, previously described in lupus patients, does also expand in severe and/or critical groups of COVID-19 patients. These DN2 cells correlate with disease severity and laboratory parameters of inflammation. However, their exact role and function in COVID-19 require to be further investigated. In this review, we highlight the DN immune responses in both rheumatic diseases and COVID-19, and we point out the importance of clarifying DN’s role in the immunopathology of the aforementioned infection, as it could probably enable better management of rheumatic diseases during the pandemic. Of note, the symptomatology of COVID-19, as well as the potential outcome of death, have given rise to a worldwide concern and scare of exposition to SARS-CoV-2, especially among the rheumatological patients who believe to be at higher risk due to their immunological background and the immunosuppressive therapies. Nevertheless, there is no convincing evidence so far that these patients are truly at higher risk than others.

Objective.To assess the efficacy and safety of the interleukin-1β (IL-1β) inhibitor canakinumab in all adolescent and adult patients with familial Mediterranean fever (FMF) identified from the Greek National Registry for off-label drug use between 2010 and 2015.Methods.In this retrospective longitudinal outcome study, clinical and laboratory data were collected from 14 patients (7 men) aged median 38.5 years (range 13–70), with median disease duration of 14 years, and active FMF despite colchicine (n = 9) or both colchicine and anakinra (n = 5).Results.All patients continued to receive canakinumab at last visit (median of 18 mos, range 13–53), which was initially given as monotherapy (n = 8) or in combination with colchicine and/or corticosteroids, every 4 (n = 7), 6 (n = 2), or 8 weeks (n = 5). Eleven patients (79%), including 6 receiving monotherapy, achieved complete clinical remission within 2 months (median), while normalization of all laboratory variables denoting inflammation occurred in 92% at 3 months (median). The remaining 3 patients achieved partial responses. Responses were sustained in all but 4 patients, who relapsed. Reducing the canakinumab administration interval from 8 or 6 weeks to 4 weeks led to suppression of disease activity in the relapsing patients. On the other hand, drug administration interval could be safely increased in 2 patients in remission. Corticosteroid doses were significantly reduced during followup. Canakinumab was well tolerated; 1 patient experienced a urinary tract infection and another one a viral gastroenteritis.Conclusion.Treatment with canakinumab in an individualized dosing scheme results in rapid and sustained remission in colchicine-resistant FMF.

Systemic Sclerosis (SSc) is an autoimmune disorder characterized by microvascular injury and diffuse fibrosis of the skin and internal organs. While macrovascular disease and higher risk for cardiovascular events are well documented in other systemic rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus, the presence and extent of atherosclerosis among patients with SSc is yet to be established. Primary cardiac involvement, due to impairment of coronary microvascular circulation and myocardial fibrosis, considerably affects prognosis and life expectancy of individuals with SSc, representing one of the leading causes of death in this population. On the other hand the existence and prevalence of atherosclerotic coronary disease remains an issue of debate as studies comparing structural and morphological markers of atherosclerosis and cardiovascular events between SSc patients and the general population have yielded controversial results. The aim of this review is to summarize recent literature about the prevalence of cardiovascular disease in SSc, review the surrogate markers of CVD that have been evaluated and examine whether common pathogenic mechanisms exist between SSc and macrovascular disease.

Analyses of the medical and economic burden of chronic disorders such as systemic lupus erythematosus (SLE) are valuable for clinical and health policy decisions. We performed a chart-based review of 215 adult SLE patients with active autoantibody-positive disease at the predefined ratio of 30% severe (involvement of major organs requiring treatment) and 70% non-severe, followed at seven hospital centres in Greece. We reviewed 318 patients consecutively registered over three months (sub-study). Disease activity, organ damage, flares and healthcare resource utilization were recorded. Costs were assessed from the third-party payer perspective. Severe SLE patients had chronic active disease more frequently (22.4% vs 4.7%), higher average SLE disease activity index (SLEDAI) (10.5 vs 6.1) and systemic lupus international collaborating clinics (SLICC) damage index (1.1 vs 0.6) than non-severe patients. The mean annual direct medical cost was €3741 for severe vs €1225 for non-severe patients. Severe flares, active renal disease and organ damage were independent cost predictors. In the sub-study, 19% of unselected patients were classified as severe SLE, and 30% of them had chronic active disease. In conclusion, this is the first study to demonstrate the significant clinical and financial burden of Greek SLE patients with active major organ disease. Among them, 30% display chronic activity, in spite of standard care, which represents a significant unmet medical need.

Background: Exercise impairment is a common symptom of systemic sclerosis (SSc), a disorder which is frequently complicated by cardiopulmonary involvement. Objectives: This study's aims were: (a) to define the prevalence and the potential causes of limited exercise capacity and (b) to study potential differences in clinical, radiological and functional characteristics and blood serology among SSc patients with exercise limitation of different etiology. Methods: Prospectively collected data on SSc patients who had conducted full lung function testing, blood serology, thorax high-resolution computed tomography, Doppler echocardiogram and a maximal cardiopulmonary exercise testing (CPET) were retrospectively analyzed. Using a CPET algorithm, patients were characterized as having normal or subnormal exercise capacity (N), respiratory limitation (RL), left ventricular dysfunction (LVD) or pulmonary vasculopathy (PV). Group comparisons were conducted using either one-way ANOVA or the Kruskal-Wallis test. A p value Results: The study population consisted of 78 patients (53.7 ± 13.7 years old; 10.3% male). PV was present in 32.1%, LVD in 25.6% and RL in 10.2%, while 32.1% of the patients constituted the N group. The presence of antisclero-70 antibodies, low anaerobic threshold and low peak exercise capacity measures could discriminate LVD from the other groups. Low end-tidal carbon dioxide pressure and its change from rest to anaerobic threshold could discriminate between the PV, LVD and N groups, while respiratory restriction along with ventilatory inefficiency indices could differentiate the RL group from the rest. Conclusions: The combined evaluation of CPET gas exchange patterns with baseline measurements could discriminate the causes of exercise limitation among SSc patients.

OBJECTIVES: To estimate the synovial membrane penetration index of celecoxib and etoricoxib, and determine their impact on pain visual analogue score (VAS). MATERIAL AND METHODS: Patients with inflammatory synovial fluid accumulation of the knee joint were randomized in three age- and gender-matched groups of 17 patients each: the celecoxib treated group, the etoricoxib treated group, and the control group. Dosages were 100mg b.i.d. for celecoxib, 90mg o.d. for etoricoxib, and no medication in the control group. The participants completed the pain VAS, and blood and synovial fluid samples were collected at baseline and seven days later at the time of Cmax for each drug. Celecoxib and etoricoxib levels were determined in serum and synovial fluid samples by UPLC coupled to ICP-MS. Identification of compounds was performed by QTOF-MS. RESULTS: After serum and synovial fluid drug concentration determination, the estimated penetration index was 23.3% (SD 32.8) for celecoxib and 49.5 % (SD 21.1) for etoricoxib, (p=0.031). In the 2 coxib groups, statistically significant reduction of pain VAS was detected (p

Background:Golimumab is a tumor necrosis inhibitor (TNFi) approved for the treatment of axial SpA (axSpA) and psoriatic arthritis (PsA), both falling under the Spondyloarthritis (SpA) domain. Real-world data regarding its effect on work productivity (WP) and activity impairment (AI) are limitedObjectives:To assess the impact of golimumab on WP and AI over 12 months of treatment in patients with SpA, overall, and in the axSpA and PsA subpopulationsMethods:A 12-month non-interventional, multicenter, prospective study performed in the routine clinical care. Data were collected at baseline (BL: prior to treatment onset), 3, 6 and 12 months. Adult work-active consented patients with axSpA [ankylosing spondylitis (AS) or non-radiographic axSpA (nr-axSpA)] or PsA, newly initiated on golimumab as per approved label, were concequetively enrolled by 20 sites. Patients prior in >1 biologic agent, or switched from another TNFi due to primary non-response or safety were excluded. WP and AI was assessed with the Work Productivity and Activity Impairment: Specific Health Problem (WPAI:SHP) instrumentResults:Between Apr-2017 and May-2018, 121 (51: PsA, 70: axSpA) eligible patients (mean age: 45.4 years; 49.6% males; 69.0% overweight/obese; median disease duration: 11.3 months), (Figure 1), were enrolled. Median study duration participation: 11.9 months. Overall, 60.3% of the patients had previously received disease-modifying antirheumatic drugs and 16.5% biologics. At BL, the mean (standard deviation: SD) DAS28-ESR of the SpA population and PsA and axSpA subpopulations was 4.0 (1.3), 4.5 (1.2), and 3.6 (1.2), while the mean (SD) BASDAI score of patients with axSpA was 5.6 (1.9). At BL 94.1 and 96.7% of the SpA population reported WP loss and AI due to their SpA respectively, and at 3 months 87.3, and 88.0% respectively. In SpA population, the median BL WP loss and AI were 70.0% and 65.0% and decreased by a median of 31.4% and 40.0% at 3 months, by 44.2% and 40.0% at 6 months and by 50.0% and 50.0% at 12 months, respectively (Table 1). Improvements in WP loss and AI were noted in patients with PsA, axSpA, AS and nr-axSpA (Table 1). 12-month golimumab retention rate: 91.7%. No new safety signals emergedTable 1.Decreases from BL at 3, 6 and at 12 months in WP loss and overall AI with the WPAI:SHP instrumentWP loss (%)AI (%)Decrease from BL,median (n)Decrease from BL,median (n)3 months6 months12 months3 months6 months12 monthsOverall SpA populationa31.4a(n=102)44.2a(n=94)50.0a(n=87)40.0a(n=107)40.0a(n=101)50.0a(n=92)PsA31.4b(n=46)51.4a(n=42)53.6a(n=40)40.0a(n=47)50.0a(n=44)60.0a(n=40)axSpA33.0b(n=56)30.4b(n=52)45.5b(n=47)40.0a(n=60)40.0b(n=57)40.0b(n=52)ASc25.1 (n=35)29.9 (n=32)39.8 (n=29)20.0 (n=39)30.0 (n=37)30.0 (n=34)nr-axSpAc47.4 (n=21)55.4 (n=20)53.2 (n=18)50.0 (n=21)55.0 (n=20)50.0 (n=18)aSignificant decreases (pbSignificant decreases (pcStatistical significance of the change from baseline was not examined due to the small observations’ numberConclusion:Patients in the SpA population and axSpA and PsA subpopulations treated with golimumab in a routine care setting experienced significant improvements in work productivity and daily activities at 3, 6 and 12 months after treatment initiationAcknowledgments:The authors thank the following investigators: Ampatziadis E., Voulgari P., Gazi S., Georgiou P., Georgountzos A., Karokis D., Mpotzoris V., Mpournazos E., Sakkas L., Sidiropoulos P., and Vassilopoulos D. The study was Sponsored by MSD, Greece.Disclosure of Interests:Panagiotis Athanassiou Grant/research support from: MSD, Genesis pharma, Janssen, Consultant of: Roche, Genesis pharma, Janssen, Speakers bureau: MSD, Janssen, Roche, Genesis pharma, Anastassios Kotrotsios Grant/research support from: MSD, Novartis, Roche, Consultant of: Bristol Myers Squibb, UCB pharma, Speakers bureau: Genesis pharma, UCB pharma, MSD, Ioannis Kallitsakis Grant/research support from: MSD, Speakers bureau: Genesis pharma, Bristol-Myers Squibb, Andreas Bounas Grant/research support from: MSD, AbbVie, Novartis, Genesis pharma, Consultant of: MSD, Bristol-Myers Squibb, UCB pharma, AbbVie, Speakers bureau: MSD, Bristol-Myers Squibb, Pfizer, Alexandros Garyfallos Grant/research support from: MSD, Aenorasis SA, Speakers bureau: MSD, Novartis, gsk, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer, GEORGIOS VOSVOTEKAS Grant/research support from: MSD, Janssen, Consultant of: MSD, Novartis, Roche, UCB pharma, Bristol-Myers Squibb, AbbVie, Speakers bureau: UCB pharma, Menarini, Bristol-Myers Squibb, MSD, Evangelia Petrikkou Employee of: MSD, Bristol Myers Squibb, Vianex SA, Gkikas Katsifis Grant/research support from: UCB Pharma, Janssen, Abbvie, Novartis, MSD, Aenorasis, Genesis Pharma, Pfizer, Roche, Consultant of: UCB Pharma, Janssen, Abbvie, Novartis, MSD, Aenorasis, Genesis Pharma, Pfizer, Roche, Speakers bureau: UCB Pharma, Janssen, Abbvie, Novartis, MSD, Aenorasis, Genesis Pharma, Pfizer, Roche

Background:Systemic autoimmune inflammatory disorders confer a higher risk of cardiovascular (CV) disease leading to increased morbidity and mortality compared to the general population. CV risk in Systemic Sclerosis (SSc) has not been studied so extensively as in other diseases, such as Rheumatoid Arthritis (RA), and the real impact of CV disease on SSc prognosis remains unknown. Surrogate markers of atherosclerosis namely carotid intima media thickness (cIMT) and pulse wave velocity (PWV) are impaired in some but not all studies in SSc patients.Objectives:The aim of the study was to investigate the prevalence of subclinical atherosclerosis assessed by cIMT and PWV between two well-characterized SSc and RA cohorts.Methods:Consecutive SSc patients attending the Scleroderma Clinic were compared with RA patients recruited in the Dudley Rheumatoid Arthritis Co-morbidity Cohort (DRACCO), a prospective study examining CV burden in RA. Cardiovascular risk was assessed using the QRisk3 and cIMT, Augmentation Index (AIx75) and central systolic and diastolic blood pressure were measured in all participants. Propensity score matching, was utilized to select patients from the two cohorts with similar demographic characteristics, CV risk factors (smoking, hypertension, obesity, dyslipidemia, diabetes) and inflammatory load. Unpaired t-test and Chi-square test of independence were applied.Results:Fifty five age- and sex-matched SSc and RA patients with similar distribution of CV risk factors were included. No difference was demonstrated between SSc and RA regarding cIMT and AIx75% (0.65 vs 0,61mm p=0,17 and 33.4 vs 31,7 p=0,397 respectively). However average QRisk3 score was significantly higher in the RA compared to the SSc group (PConclusion:The results of this comparative study show that subclinical atherosclerosis is comparable between individuals with SSc and RA, a systemic disease with well-defined high atherosclerotic burden. RA patients have higher CV risk (QRisk3 algorithm) suggesting that disease-specific factors such chronic high-grade inflammation may influence the CV risk in this population.References:[1]Ozen G, et al. Subclinical Atherosclerosis in Systemic Sclerosis: Not Less Frequent Than Rheumatoid Arthritis and Not Detected With Cardiovascular Risk Indices. Arthritis Care Res (Hoboken) 2016; 68:1538-46[2]Pagkopoulou E, et al., Cardiovascular risk in systemic sclerosis: Micro- and Macro-vascular involvement. Indian J Rheumatol 2017;12: 211-7Table 1.Demographic and cardiovascular risk factors of the matched patientsRASScPN=55N=55Age63.6 (14.8)61.3 (10.9)0.140Female49 (89.1%)53 (96.4%)0.438Smoking10 (18.2%)13 (23.6%)0.5Diabetes0 (0.00%)1 (1.82%)0.364Hyperlipidemia:7 (12.7%)6 (10.9%)1.000Hypertension:23 (41.8%)19 (34.5%)0.441ESRD20.4 (18.4)22.0 (19.1)0.666CRP8.38 (11.6)6.65 (30.2)0.692Table 2.Comparison of IMT, AIx75, Framingham and QRISK3 between matched patientsRASScPN=55N=55IMT right average0.65 (0.17)0.61 (0.12)0.175IMT left average0.67 (0.15)0.64 (0.13)0.214IMT average0.66 (0.14)0.63 (0.10)0.137AIX 75% (%)33.4 (9.23)31.7 (10.8)0.397Framingham risk< 0.001< 10%9 (31.0%)37 (74.0%)10 – 20%12 (41.4%)9 (18.0%)20 – 30%3 (10.3%)4 (8.00%)>30%5 (17.2%)0 (0.00%)QRISK318.2 (15.3)11.1 (10.6)0.006Disclosure of Interests:Pantelis Baniotopoulos: None declared, Eleni Pagkopoulou: None declared, Stergios Soulaidopoulos: None declared, Aamer Sandoo: None declared, Niki Katsiki: None declared, Asterios Karagiannis: None declared, Karen Douglas: None declared, Alexandros Garyfallos Grant/research support from: MSD, Aenorasis SA, Speakers bureau: MSD, Novartis, gsk, Georeg Kitas: None declared, Theodoros Dimitroulas: None declared

Background:Comorbidities in rheumatic diseases (RDs) have been associated with increased morbidity and mortality. Evidence on prevalence of comorbidities in antiphospholipid syndrome (APS) and its difference from high comorbidity burden RDs is limited.Objectives:To compare the prevalence of common comorbidities between APS [primary (PAPS) and Systemic lupus erythematosus (SLE)-APS] and Rheumatoid arthritis (RA) patients.Methods:326 APS patients from the Greek registry (237 women, mean age 48.7±13.4 years, 161 PAPS) were matched 1:2 for age and sex with 652 RA patients from Greek RA Registry. Prevalence of cardiovascular (CV) risk factors, stroke, coronary artery disease (CAD), osteoporosis, diabetes mellitus (DM), Chronic obstructive pulmonary disease (COPD), depression and neoplasms were compared between APS and RA using logistic regression analysis.Results:Regarding CV burden, hyperlipidemia and obesity (ΒMI≥30) were comparable while hypertension, smoking, CAD and stroke were more prevalent in APS compared to RA patients (Table 1). Osteoporosis and depression were more frequent in APS while DM, COPD and neoplasms were comparable between two groups. Comparison of APS subgroups to 1:2 matched RA patients revealed that smoking and stroke were more prevalent in PAPS and SLE-APS vs RA. Hypertension, CAD and osteoporosis were more prevalent only in SLE-APS vs. RA while DM was less prevalent in PAPS vs. RA patients.Table 1.Comparison of comorbidities between Antiphospholipid syndrome (APS) vs. matched Rheumatoid Arthritis (RA) patients and between primary APS (PAPS) or Systemic Lupus Erythematosus-APS (SLE-APS) vs matched RA patientsAPSRAOR*PAPSRAORSLE-APSRAORn (%)326652161322165330Hypertension97 (29.8)136 (21)1.61 (1.19-2.18)40 (25)75 (23.3)1.09 (0.70-1.69)57 (34.6)61 (18.5)2.33 (1.52-3.56)Smoking175 (53.7)264 (40.5)1.70 (1.30-2.22)87 (54)142 (44)1.49 (1.02-2.18)88 (53.3)122 (37)1.95 (1.33-2.85)Hyperlipidemia79 (24.2)135 (20.7)1.23 (0.89-1.68)40 (24.8)62 (19.3)1.39 (0.88-2.18)39 (23.6)73 (22)1.09 (0.70-1.70)Obesity48 (20.5)105 (19.5)1.06 (0.73-1.56)20 (17)51 (19)0.86 (0.49-1.52)28 (24)54 (19.7)1.28 (0.76-2.15)Stroke±66 (20.3)9 (1.4)13.8 (6.5-29.1)36 (22.4)4 (1.2)19.9 (6.6-59.9)30 (18.2)5 (1.5)7.8 (2.7-22.6)Coronary disease±16 (4.9)13 (2)3.14 (1.17-8.45)2 (1.2)7 (2.2)0.46 (0.04-4.77)14 (8.5)6 (1.8)10.9 (2.7-44.3)Osteoporosis×66 (20.3)92 (14)1.45 (1.01-2.06)19 (11.8)42 (13)0.96 (0.54-1.73)47 (28.5)50 (15)1.91 (1.20-3.05)Diabetes×18 (5.5)58 (9)0.58 (0.33-1.01)5 (3)29 (9)0.34 (0.13-0.89)13 (8)29 (9)0.88 (0.44-1.79)COPD≠11 (3.4)14 (2.2)1.26 (0.56-2.84)3 (1.9)6 (2)0.96 (0.23-4.0)8 (5)8 (2.4)1.28 (0.44-3.72)Depression#53 (16.3)66 (10)1.70 (1.15-2.53)23 (14)30 (9.3)1.69 (0.93-3.05)30 (18.2)36 (10.9)1.65 (0.96-2.84)Neoplasms˅14 (4.3)27 (4.1)1.05 (0.54-2.06)5 (3)12 (3.7)0.84 (0.28-2.52)9 (5.5)15 (4.6)1.31 (0.55-3.1)*OR: Odds ratio, crude or adjusted for: ± age, sex, smoking, hypertension, hyperlipidemia, BMI, corticosteroid (Cs) duration × Cs duration ≠ smoking, Cs duration #sex, disease duration, Cs duration ˅ age, disease durationConclusion:Comorbidity burden in APS (PAPS and SLE-APS) is comparable or even higher to that in RA, entailing a high level of diligence for CV risk prevention, awareness for depression and corticosteroid exposure minimization.Disclosure of Interests:Stylianos Panopoulos: None declared, Konstantinos Thomas: None declared, Georgios Georgiopoulos: None declared, Dimitrios Boumpas Grant/research support from: Unrestricted grant support from various pharmaceutical companies, Christina Katsiari: None declared, George Bertsias Grant/research support from: GSK, Consultant of: Novartis, Alexandros Drosos: None declared, Kyriaki Boki: None declared, Theodoros Dimitroulas: None declared, Alexandros Garyfallos Grant/research support from: MSD, Aenorasis SA, Speakers bureau: MSD, Novartis, gsk, Charalambos Papagoras: None declared, PELAGIA KATSIMPRI: None declared, Apostolos Tziortziotis: None declared, Christina Adamichou: None declared, Evripidis Kaltsonoudis: None declared, Evangelia Argyriou: None declared, GEORGIOS VOSVOTEKAS Grant/research support from: MSD, Janssen, Consultant of: MSD, Novartis, Roche, UCB pharma, Bristol-Myers Squibb, AbbVie, Speakers bureau: UCB pharma, Menarini, Bristol-Myers Squibb, MSD, Petros Sfikakis Grant/research support from: Grant/research support from Abvie, Novartis, MSD, Actelion, Amgen, Pfizer, Janssen Pharmaceutical, UCB, Dimitrios Vassilopoulos: None declared, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer

Background:In systemic sclerosis (SSc), inflammation and microvascular damage are fundamental in the progressive fibrotic process. Although the presence of accelerated atherosclerosis in SSc is not as well-described as in other systemic disorders namely rheumatoid arthritis, it appears that individuals suffering from the disease are at higher risk for cardiovascular events. Nailfold Video Capillaroscopy (NVC) is a non-invasive and reproducible imaging technique of the capillary vascular bed, used in the evaluation of microvascular involvement in SSc. Previous data on the association between micro- and macrovascular damage are scarce.Objectives:The aim of this study was to examine the association between micro- and macrovascular involvement in patients with SSc.Methods:This is a cross-sectional study including consecutive SSc patients attending to a Scleroderma Outpatient Clinic between March and September 2018. All the study participants underwent NVC and the findings were classified in one of the following qualitative patterns: early, active, and late NVC pattern. Capillary’s density was evaluated in the distal row of each finger, based on the number of capillaries per 1 mm and the mean capillaroscopic skin ulcer risk index (CSURI) was automatically calculated with software image analysis. Carotid intima-media thickness (cIMT) was measured in the common carotid artery bilaterally, according to the relevant guidelines. Aortic blood pressure (BP), heart rate adjusted augmentation index [AIx(75)] and carotid-femoral pulse wave velocity (PWV) were evaluated with applanation tonometry (Sphygmocor).Results:Sixty-four (95,3% women) SSc individuals with mean age 57.54±12.99 years were included in this analysis. AIx(75) was significantly associated with CSURI (r=0.261; p=0.038) and inversely associated with the number of capillaries (r=-0.271; p=0.030) suggesting a link between the degree of microvascular disease and arterial stiffening. Regarding SSc-specific NVC patterns, AIx(75) were marginally lower in patients with early compared to active or late patterns (25.95±11.27 vs 32.50±11.17 vs 31.62±10.32%; p=0.081 and p=0.083) confirming a trend between progressive microvasculopathy and arterial stiffness. Mean cIMT was negatively correlated with enlarged capillary loops. Brachial or aortic systolic BP (SBP) and pulse pressure (PP) levels were not correlated with any of the studied NVC parameters.Conclusion:Microvascular vasculopathy is associated with higher wave reflections, indicating an association between atherosclerotic disease and microvascular injury in SSc patients. Such observations may provide possible explanations for the excessive cardiovascular and mortality risk in this population.References:[1]Soulaidopoulos, S., Pagkopoulou, E., Katsiki, N. et al. Arterial stiffness correlates with progressive nailfold capillary microscopic changes in systemic sclerosis: results from a cross-sectional study. Arthritis Res Ther 21, 253 (2019)[2]Jung K-H, Lim MJ, Kwon SR, Kim D, Joo K, Park W. Nailfold capillary microscopic changes and arterial stiffness in Korean systemic sclerosis patients. Mod Rheumatol. 2015;25:328–31.[3]Aviña-zubieta JA, Man A, Yurkovich M, Huang K, Sayre EC. Early cardiovascular disease after the diagnosis of systemic sclerosis. Am J Med. 2015;129:324–31Disclosure of Interests:Eleni Pagkopoulou: None declared, Stergios Soulaidopoulos: None declared, Eva Triantafyllidou: None declared, Niki Katsiki: None declared, Georeg Kitas: None declared, Asterios Karagiannis: None declared, Alexandros Garyfallos Grant/research support from: MSD, Aenorasis SA, Speakers bureau: MSD, Novartis, gsk, Theodoros Dimitroulas: None declared

Background:Despite the increased incidence of influenza infection in rheumatoid arthritis (RA) patients, vaccination coverage has been shown to be suboptimal. Prospective data regarding the current rate and predictors of influenza vaccination adherence in RA patients are limited.Objectives:To calculate the current rate and predictors of influenza vaccination in a real-life, prospective, longitudinal RA cohort.Methods:Data regarding demographics, disease characteristics, treatments and co-morbidities from a multi-center, longitudinal cohort of Greek RA patients were collected at baseline and ~ 3 years later. Disease and patient characteristics were compared between patients with at least one influenza vaccine administration and non-vaccinated ones, during the 3 year follow-up period.Results:From a cohort of 1,569 RA patients, 1,406 with available vaccination data at baseline and 3 years later (mean interval: 2.9 years) were included; (women: 80.4%, mean age: 61.8 years, mean disease duration: 9.7 years, RF and/or anti-CCP positive: 50.4%, mean DAS-28 = 3.33, mean HAQ: 0.44, bDMARD use: 44.8%). At baseline, 54.2% of patients reported influenza vaccination in the past (31.8% during the previous season), while during the 3 year follow-up period, 81% had ≥1 influenza vaccinations (p=Conclusion:In this ongoing, longitudinal, prospective, real-life RA cohort study, a significant increase in the influenza vaccination coverage was noted (from 53% to 81%). Influenza vaccination was independently associated with recent history of influenza vaccination, older age, and bDMARD treatment.Acknowledgments:Supported by grants from the Greek Rheumatology Society and Professional Association of Rheumatologists.Disclosure of Interests:Konstantinos Thomas: None declared, Argyro Lazarini: None declared, Evripidis Kaltsonoudis: None declared, Alexandros Drosos: None declared, ARGYRO REPA: None declared, Prodromos Sidiropoulos: None declared, Kalliopi Fragkiadaki: None declared, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer, Petros Sfikakis Grant/research support from: Grant/research support from Abvie, Novartis, MSD, Actelion, Amgen, Pfizer, Janssen Pharmaceutical, UCB, Panagiota Tsatsani: None declared, Sousana Gazi: None declared, Pelagia Katsimbri: None declared, Dimitrios Boumpas: None declared, Evangelia Argyriou: None declared, Kyriaki Boki: None declared, Gerasimos Evangelatos: None declared, Alexios Iliopoulos: None declared, Konstantina Karagianni: None declared, Lazaros Sakkas: None declared, Konstantinos Melissaropoulos: None declared, Panagiotis Georgiou: None declared, Eleftheria Grika: None declared, PANAYIOTIS VLACHOYIANNOPOULOS: None declared, Theodoros Dimitroulas: None declared, Alexandros Garyfallos Grant/research support from: MSD, Aenorasis SA, Speakers bureau: MSD, Novartis, gsk, Constantinos Georganas: None declared, Periklis Vounotrypidis: None declared, Konstantinos Ntelis: None declared, Maria Areti: None declared, George D Kitas: None declared, Dimitrios Vassilopoulos: None declared

The term “age-associated B cells” (ABCs) refers to a heterogeneous B cell subset (CD19+,CD21−, CD11c+,T-bet+) which is expanded in the elderly, but also accumulates prematurely in patients with autoimmune disorders and/or infectious diseases. In healthy individuals, ABCs represent a low prevalence population that positively impacts immunosenescence. In autoimmunity and infections though, ABCs expand dramatically and produce high titers of antibodies, thus playing a role in the regulation of humoral responses. Despite the fact that these observations were made on both mice and humans, the functional features of ABCs and their exact role in human health and disease are still elusive. This review focuses on ABC and ABC-like sub-populations found in Systemic Lupus Erythematosus (SLE) patients (such as the double negative 2;DN2 population: CD19+,IgD−,CD27−, CXCR5−,T-bet+) and broaches the subject of their potential use as prognostic and/or diagnostic markers. The identification of novel biomarkers, via correlating the cell populations with the clinical profile of the patients, should enable better patient stratification and monitoring. Moreover, the necessity and importance of elucidating the role of transcription factor T-bet (TBX21) in the pathogenesis of human autoimmunity are addressed. T-bet, whose expression is upregulated in both mouse and human ABCs, is considered to play a major role in various aspects of autoimmunity, such as the production of autoreactive IgG, the enhanced antigen presentation to T cells and also the formation of spontaneous germinal centres (GC). Shedding light to its role in human disease, in conjunction with the characterisation of genes and pathways associated with the transcription factor itself, may lead to the discovery of novel druggable targets.

Background A number of cross-sectional studies have shown that approximately one quarter of rheumatoid arthritis (RA) patients are being treated with biologic disease modifying anti-rheumatic drugs (bDMARDs) as monotherapy. Data regarding the retention of bDMARD monotherapy in real-life settings are limited. Objectives To study the survival rate of bDMARD monotherapy in RA patients in daily clinical practice. Methods Multicenter (11 hospital, 3 private office practices), prospective, RA epidemiological study in Greece. At baseline and after one year of follow-up, demographics, disease characteristics, treatments, co-morbidities and serious events (serious infections, cardiovascular events, neoplasms, osteoporotic fractures) were collected via a web-based platform. Results 1.323 RA patients with paired evaluations one year apart (mean interval: 13.2±3.7 months) were included. Among 611 bDMARD treated patients, 155 patients (25%) were on bDMARD monotherapy (women: 87%, mean age: 60.4 years, mean disease duration: 15 years, RF and/or anti-CCP positive: 66%, TNFi therapy: 57%). The majority had been previously on and had discontinued their csDMARDs (90%). During follow-up, 15% (n=24) discontinued their bDMARD; most of them stayed off any type of therapy (83%) while the rest continued with synthetic DMARD (csDMARD) monotherapy (17%). From the remaining 131 patients, 96 (73%) remained on bDMARD monotherapy (85%, n=82 on the same biologic) while in 27% (n=35) a csDMARD was added. Serious events occurred in 7.7% of patients (n=12). Overall, at the end of 1st year, approximately half of patients (53%, n=82) remained on their initial bDMARD monotherapy. Factors associated with continuation of the same bDMARD by multivariate analysis were a low HAQ score (OR=0.48, 95% C.I.=0.23–0.99, p=0.047) and corticosteroid use (OR=2.2, 95% C.I.=1.02–5.1, p=0.044) at baseline as well as the absence of a serious event during the 1st year of follow-up (OR=0.14, 95% C.I.=0.016–1.3, p=0.094). Conclusions In real life settings, only half of patients who are on bDMARD monotherapy continue the same agent one year later. Low HAQ score, corticosteroid use and absence of a serious event during therapy predicted bDMARD monotherapy survival. Acknowledgements Supported by grants from the Greek Rheumatology Society and Professional Association of Rheumatologists. Disclosure of Interest None declared

Background It is unclear if the widespread use of biologic DMARDs (bDMARDs) and the implementation of the treat to target approach have led to better disease control in patients with rheumatoid arthritis (RA) in daily clinical practice. Objectives To study the longitudinal changes in disease activity in a large, real life, longitudinal RA cohort. Methods Multicenter (11 hospitals, 3 private offices), prospective, RA epidemiological study in Greece. Demographics, disease characteristics, treatments and co-morbidities were collected via a web-based platform in registered patients at baseline and one year after their 1st visit. Results 1.096 RA patients with available paired evaluations one year apart (mean interval: 13.4±3.6 months) were included (women: 78%, mean age: 62.8 years, mean disease duration: 11 years, RF and/or anti-CCP positive: 60%, mean HAQ: 0.44±0.56). At baseline, 50% (n=548) of patients were on conventional DMARDs (csDMARDs) alone, 35% on cs- and b-DMARD combination (n=379) and 11% on bDMARD monotherapy (n=124). Among bDMARD treated patients, 60% were receiving tumour necrosis factor inhibitors (TNFi) while 40% were on corticosteroids (mean daily dose: 4.7 mg). Despite these therapies, 43% of patients had active disease (DAS28-ERS>3.2); 34% moderate (MDA, DAS28-ESR=3.2–5.1) and 9% high (HDA, DAS28-ESR>5.1) disease activity. During the 1 year observation period, among the group of patients with MDA who were only on csDMARDs, 15% started a bDMARD while among those on bDMARDs, 11% switched to another bDMARD. The respective rates of starting a bDMARD (in those on csDMARDs) or switching to another bDMARD (in those on bDMARDs), were much higher for patients in the HDA group (41% and 32% respectively, p Conclusions In a large, real life, RA cohort with almost half of patients on bDMARD-based therapies, more than one third of patients had still active disease at the end of the 1st year of follow-up. These findings could be explained in part by the low rate of bDMARD initiation or switching in this cohort or they could indicate the limitations of current therapeutic approaches in RA patients with longstanding disease in Greece. Acknowledgements Supported by grants from the Greek Rheumatology Society and Professional Association of Rheumatologists. Disclosure of Interest None declared

Background Juvenile Idiopathic Arthritis (JIA) is the most common rheumatic disease in childhood and affects negatively both physical and psychosocial functioning. Objectives To explore the long-term impact of JIA on quality of life of adult patients in Northern Greece. Methods Adult patients with a definite diagnosis of JIA were assessed by the SF-12v2 questionnaire in the outpatient transition (from Paediatric to adult Rheumatology) clinic of Hippokratio Hospital of Thessaloniki. SF-12 questionnaire is a quality of life assessment tool and consists of a Physical Component Summary (PCS) and a Mental Component Summary (MCS). PCS and MCS of the patient group were compared to an age-matched control group, using t-test or Mann-Whitney U test, as appropriate. Moreover, percentages of patients and controls who were severely affected ( Results A total of 50 patients and 135 controls were enrolled in the study. The median (IQR) patient age was 3210 years and disease duration was 249,5 years. PCS scores of patients group were statistically significantly lower compared to control group (p=0.021) and more patients than controls scored low values ( Conclusions There is an apparent impact of JIA on many patients‘ quality of life, specifically in terms of their physical health, that persists for many years after disease onset, which is in line with studies from other countries. This could be related to disease severity, disease subtype and duration, socioeconomic status and availability of treatment options. Interestingly, JIA wasn’t found to affect the patients’ mental health. A more specific psychometric test would be appropriate for in-depth analysis and confirmation of this result. Study design did not allow subgroup analysis according to JIA subtype, disease severity or duration, highlighting the need for long-term outcome studies focusing on the risk factors which may be involved. References [1] Foster H, et al. Outcome in adults with juvenile idiopathic arthritis: A quality of life study. Arthritis & Rheumatism. 2003;48(3):767–775 [2] Kontodimopoulos N, et al. Validity of SF-12 summary scores in a Greek general population. Health Qual Life Outcomes. 2007Sep 28;5:55 [3] Minden K, et al. Long-term outcome in patients with juvenile idiopathic arthritis. Arthritis & Rheumatism. 2002;46(9):2392–2401 [4] Anink J, et al. Long-term quality of life and functional outcome of patients with juvenile idiopathic arthritis in the biologic era: a longitudinal follow-up study in the Dutch Arthritis and Biologicals in Children Register. Rheumatology. 2015;54(11):1964–1969 Disclosure of Interest None declared

Rheumatoid arthritis (RA) is a chronic inflammatory systemic disease which commonly requires treatment with biologic agents targeting various inflammatory pathways. Tumor necrosis factor alpha is a proinflammatory cytokine which plays a pivotal role not only in the pathogenesis of RA but also in other autoimmune diseases such as primary biliary cirrhosis. The co-existence of more than one autoimmune disorder in the same individual is very challenging in the daily practice as therapy strategies applicable to one disease setting may cause clinical and/or biochemical relapse of the other clinical entity. As a result, treatment options able to control different diseases are highly desirable among rheumatologists and other specialties. In that respect, we present a case of a 61-year-old female patient with RA and concomitant primary biliary cirrhosis with poor clinical response to conventional disease-modifying drugs for RA. The introduction of tumor necrosis factor alpha antagonist infliximab led to significant clinical improvement of RA and to stabilization of liver function. In this case review study, we discuss aspects of pathophysiology of primary biliary cirrhosis associated with tumor necrosis alpha and we review the available data of similar published cases.

Background There are limited literature data regarding the characteristics of rheumatoid arthritis (RA) patients treated with biologic DMARD (bDMARD) monotherapy. Objectives To evaluate the disease and treatment characteristics of RA patients treated with bDMARD monotherapy. Methods Multicenter, cross-sectional RA epidemiological study in Greece (06/2015–05/2016, ERE RA Study Group). Demographics, disease characteristics, treatment and co-morbidity data were collected via a web-based platform Results 1036 RA patients treated with bDMARDs were identified during the one year recruitment period (female: 82%, mean age: 61.5±13 years, mean disease duration: 12.5±8.9 years, mean DAS28-ESR: 3.4±3.3). Among them, 26% (n=273) were receiving bDMARDs as monotherapy and 8% (n=23) of them had never tried conventional synthetic DMARDs (csDMARDs) before; The latter group (n=23) compared to the csDMARD-exposed (92%, n=250) group, had more often co-morbidities [cardiovascular disease (22% vs. 8%, p=0.029), chronic hepatitis (13% vs. 3%, p=0.02), hypertension (57% vs. 38%, p=0.08), COPD (13% vs. 4.4%, p=0.07)] or were active smokers (41% vs. 14%, p=0.001). csDMARD discontinuation was mainly due to adverse events (AEs, 58%) followed by inadequate response (IR, 44%). Compared to the cs- and b-DMARD combination therapy group, monotherapy treated patients were more frequently seropositive (64% vs. 57%, p=0.003), had lower DAS28-ESR (3.2 vs 3.5, p=0.009) and were more likely to have discontinued csDMARDs for AEs (58% vs. 21%, p Conclusions In our large RA cohort, one out of four bDMARD treated patients, were receiving bDMARDs as monotherapy. Co-morbidities rather than RA characteristics influence the initial decision for bDMARD monotherapy whereas among those starting combination cs- and b-DMARD therapy, the majority discontinue csDMARDs due to AEs. Acknowledgements Grant support from the Hellenic Rheumatology Society and Professional Association of Rheumatologists. Disclosure of Interest None declared