Patients with immune-mediated rheumatic disease-related calcinosis comprise a subgroup at risk of encountering a more severe clinical outcome. Early assessment is pivotal for preventing overall disease progression, as calcinosis is commonly overlooked until several years into the disease and is considered as a ‘non-lethal’ manifestation. This single-center retrospective study explored the prevalence, clinical associations, and impact on survival of subcutaneous calcinosis in 86 patients with immune-mediated rheumatic diseases (IMRD). Calcinosis predominantly appeared in individuals with longstanding disease, particularly systemic sclerosis (SSc), constituting 74% of cases. Smaller calcinosis lesions (≤1 cm) were associated with interstitial lung disease, musculoskeletal involvement, and digital ulcerations, while larger lesions (≥4 cm) were associated with malignancy, severe peripheral artery disease, and systemic arterial hypertension. The SSc calcinosis subgroup exhibited a higher mean adjusted European Scleroderma Study Group Activity Index score than those without. However, survival rates did not significantly differ between the two groups. Diltiazem was the most commonly used treatment, and while bisphosphonates reduced complications related to calcinosis, complete resolution was not achieved. The findings underscore current limitations in diagnosing, monitoring, and treating calcinosis, emphasizing the need for further research and improved therapeutic strategies to improve patient care and outcomes.

ObjectiveThe randomised placebo-controlled GLORIA (Glucocorticoid LOw-dose in RheumatoId Arthritis) trial evaluated the benefits and harms of prednisolone 5 mg/day added to standard care for 2 years in patients aged 65+ years with rheumatoid arthritis (RA). Here, we studied disease activity, flares and possible adrenal insufficiency after blinded withdrawal of study medication.MethodsPer protocol, patients successfully completing the 2-year trial period linearly tapered and stopped blinded study medication in 3 months. We compared changes in disease activity after taper between treatment groups (one-sided testing). Secondary outcomes (two-sided tests) comprised disease flares (DAS28 (Disease Activity Score 28 joints) increase >0.6, open-label glucocorticoids or disease-modifying antirheumatic drug (DMARD) increase/switch after week 4 of tapering) and symptoms/signs of adrenal insufficiency. In a subset of patients from 3 Dutch centres, cortisol and ACTH were measured in spot serum samples after tapering.Results191 patients were eligible; 36 met treatment-related flare criteria and were only included in the flare analysis. Mean (SD) DAS28 change at follow-up: 0.2 (1.0) in the prednisolone group (n=76) vs 0.0 (1.2) in placebo (n=79). Adjusted for baseline, the between-group difference in DAS28 increase was 0.16 (95% confidence limit –0.06, p=0.12). Flares occurred in 45% of prednisolone patients compared with 33% in placebo, relative risk (RR) 1.37 (95% CI 0.95 to 1.98; p=0.12). We found no evidence for adrenal insufficiency.ConclusionsTapering prednisolone moderately increases disease activity to the levels of the placebo group (mean still at low disease activity levels) and numerically increases the risk of flare without evidence for adrenal insufficiency. This suggests that withdrawal of low-dose prednisolone is feasible and safe after 2 years of administration.

Objective To assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice. Methods We performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab. Results 254 patients were treated with rituximab, in 58% for lung and in 32% for skin involvement. After a median follow-up of 2 years, about 70% of the patients had no side effect. Comparison of treated patients with 9575 propensity-score matched patients showed that patients treated with rituximab were more likely to have skin fibrosis improvement (22.7 vs 14.03 events per 100 person-years; OR: 2.79 [1.47-5.32]; p=0.002). Treated patients did not have significantly different rates of decrease in forced vital capacity (FVC)>10% (OR: 1.03 [0.55-1.94]; p=0.93) nor in carbon monoxide diffusing capacity (DLCO) decrease. Patients having received rituximab were more prone to stop or decrease steroids (OR: 2.34 [1.56-3.53], p<0.0001). Patients treated concomitantly with mycophenolate mofetil had a trend for better outcomes as compared with patients receiving rituximab alone (delta FVC: 5.22 [0.83-9.62]; p=0.019 as compared with controls vs 3 [0.66-5.35]; p=0.012). Conclusion Rituximab use was associated with a good safety profile in this large SSc-cohort. Significant change was observed on skin fibrosis, but not on lung. However, the limitation is the observational design. The potential stabilisation of lung fibrosis by rituximab has to be addressed by a randomised trial.

Rheumatoid arthritis (RA) is a chronic inflammatory disease of the joints affecting 1% of the world population. It has major impact on patients through disability and associated comorbidities. Current treatment strategies have considerably improved the prognosis, but recent innovations (especially biologic drugs and the new class of so-called “JAK/STAT inhibitors”) have important safety issues and are very costly. Glucocorticoids (GCs) are highly effective in RA, and could reduce the need for expensive treatment with biologic agents. However, despite more than 65 years of clinical experience, there is a lack of studies large enough to adequately document the benefit/harm balance. The result is inappropriate treatment strategies, i.e. both under-use and over-use of GCs, and consequently suboptimal treatment of RA.

We hypothesized that clinical outcomes might be influenced by personality type (A, B, C, D) in rheumatoid arthritis (RA) and ankylosing spondylitis (AS). One hundred ninety-four patients (104 with RA, 90 with AS) participated in a questionnaire study. We evaluated health-related quality of life (HRQoL) using the Medical Outcome Study Short-Form 36 (SF-36), personality type A/B with the Jenkins Activity Survey, type C with the State-Trait Anger Expression Inventory Anger-in Scale, type D with the Type D Personality Scale, and disease activity with Disease Activity Score with 28 joints for RA and Bath Ankylosing Spondylitis Disease Activity Index for AS. We used Pearson's correlation coefficient, independent samples t tests, and multivariate analyses of variance. In the RA group, type D personality was significantly correlated with 7/12 SF-36 components. AS patients with type D personality had deficits in all SF-36 subscales. Type D was related with higher disease activity in RA and AS. Both RA and AS type C patients had more active disease forms and negatively affected HRQoL subscales. In the RA group, type A personality did not correlate with HRQoL, but it positively influenced pain visual analog scale scores. In AS patients, type A personality was linked with higher HRQoL and with less active disease. Type C and type D personality types were correlated with decreased HRQoL and higher disease activity in RA and AS patients. Type A personality was associated with less active disease and higher HRQoL in AS patients and with less pain in RA patients.

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

Background. Sjögren syndrome (SS) is a systemic chronic inflammatory disorder characterized by lymphocytic infiltrates in exocrine organs. Ultrasonography (US) demonstrates specificity and sensibility in major salivary glands (SG) evaluation. Recent data confirm US might be used as primary evaluation technique for its ability to show structural alterations of parenchyma (1). Objective. To assess the gray scale (GS) parenchymal inhomogeneity of major SG in patients with established primary and secondary SS and correlate with clinical and biological data. Methods. Consecutive patients with SS were recruited and SG US was performed. Inhomogeneity of glandular parenchyma was quantified binary on each gland. ESSDAI and ESSPRI scores were calculated. Statistics was performed with SPSS. Results. Twenty one (42.85% primary SS, 90.47% female) consecutive patients were included. Mean age was 53.66+/-12.99 years and disease duration 5.33+/-3.74 years. Antibody SSA/SSB presence was found in 85.7% (18/21). ESSDAI mean was 8.67+/-8.9 (0-29), ESSPRI 10.13+/-5.59(0-20). There were no differences regarding ESSDAI and ESSPRI in the two groups (primary and secondary SS). Right parotid gland showed alterations in 71.4% patients (77% with primary SS, 66% with secondary SS). Frequently inhomogeneity was found in all major SG (33%, 22% left and right submandibular, 77%, 44.4% left and right parotid glands) in primary SS. Both submandibular glands were symmetrically involved (p<0.02). Duration of disease was negatively correlated to inhomogeneity of right parotid gland (p<0.02). Conclusion. Inhomogeneity in major SG in GS US was found in the majority of patients with primary and secondary SS. The symmetrical involvement of submandibular glands was significant. The inhomogeneity appears in the early period of diagnosis. No major differences were found between two groups.

Objectives. The objectives of this study were to evaluate the prevalence of disease severity in PsA and to assess the factors that might explain it. Methods. This was a cross-sectional study of unselected PsA patients. Severity was defined according to GRAPPA criteria of severity. Factors potentially associated with severity (demographical, clinical, laboratory variables, treatment related factors and comorbidities) were assessed by uni- and multivariate logistic regressions. Results. A total of 129 PsA patients were analysed: 77 (59.7%) women, mean±standard deviation age 53.5±11.8 years, and mean disease duration 7±7.4 years. Twenty-for patients (18.6%) had severe PsA. In the univariate regression, disease severity was associated with psoriasis duration, PsA duration, current moderate/severe skin disease, nail disease, history of corticotherapy, and total number of previous synthetic and biologic DMARDs. In the multivariate analysis, PsA severity was explained by the presence of current moderate/severe psoriasis – odds ratio 5.88 (95% confidence interval 1.39; 25.00) and history of corticosteroids – 4.65 (1.13; 18.87). Conclusion. PsA severity is best explained by the presence of moderate or severe psoriasis and past treatment with corticosteroids, but further longitudinal studies are needed to identify predictive factors.

Background Corticosteroids are still one of the main treatment in Systemic Lupus Erythematosus (SLE). Beside the effect on controlling disease activity, they are also implicated in damage accrual. Both patients and physicians are some time afraid to adopt a steroid free regimen when possible. Objectives To evaluate the knowledge and perception of patients with SLE upon glucocorticoids. Methods 84 patients with SLE were evaluated and data about demographic, clinical, serological characteristics or treatment were collected. Presence of steroids related side effects like hypertension, osteoporosis, cataracts or diabetes mellitus were also assessed. All patients completed a questionnaire in order to evaluate patient9s knowledge about steroids. They were asked if they had a discussion with the doctors about corticotherapy and side effects related to them, if they consider that this treatment could be stopped with specialist approval. Statistics was performed with SPSS program. Results All patients had treatment with corticosteroids during disease evolution. 57.14% of them experienced at least one steroids related side effect. This patients were significant older: mean age at evaluation 49.50 versus 36.47 (p<0.0001), had a longer disease duration: mean SLE duration 9.27 versus 4.69 (p0.016), a higher mean Prednisone equivalent dose: 8.86 versus 4.71 (p 0.031), a higher mean SLICC Damage Index: 1.53 versus 0.44 (p 0.001) than patients without steroids related side effects. This complications were significantly more rare in patients that were on a steroid free regimen at the moment of evaluation versus those on a continuum steroid regimen (7.14% versus 50%, p<0.0001). When patients were asked if they will stop steroids according to medical advice, almost 1/3 of patients - 28.57% - responded “no- to afraid to do that”. Patients willingness to adhere to a steroid free regimen in the future according to a physician recommendation was significant more frequent in younger patients (p 0.031, r -0.235), in those with steroids initiated in less than 1 year (p 0.016, r -0.297) and in those with less damage accrual (p 0.017, r-0.267). Flare at the moment of evaluation significantly reduced this possibility, at least from the patient perspective (p0.041, r 0.224). The likelihood of a future steroid free regimen was increased by a previous discussion patient-doctor about steroids (p0.002). Conclusions This study clearly shows that an open discussion with our SLE patients about corticosteroids is mandatory from the beginning. Patients should be informed about possibility of a steroid free regimen when disease status permits. This will increase patient willingness to get free of steroids when possible, helping physician to limit the continuum damage accrual of SLE patients. References Duru N, van der Goes MC, Jacobs JWG et al, “ EULAR evidence-based and consensus-based recommendations on the management of medium to high-dose glucocorticoid therapy in rheumatic diseases”, Ann Rheum Dis 2013; 72: 1905–1913. Disclosure of Interest None declared

Background The Quality of life (QoL) in patients with SLE is deeply affected by multiple factors including psychological and physical factors, the functional status and the general perception over health. Objectives Evaluation of Quality of life and the perceptions over the disease in patients with SLE.functional status and the general perception over health. Methods This is a 10 month prospective, cross-sectional study performed on 52 patients hospitalized diagnosed with SLE according to SLICC 2012 criteria. There were evaluated demographic data, organs manifestations, disease activity scores (SLEDAI, SLICC) and treatment. To evaluate the QoL several questionnaires were performed: Health Assessment Questionnaire (HAQ), EuroQol five dimensions questionnaire (EQ5d), Illness Perception Questionnaire and SF-36. Results All patients were women with the mean age 49 years with a mean durations of illness 136 month (14). The mean SLEDAI – 5,52 (5,37) and SLICC – 2,25 (1,71). The mean HAQ value was 0,83 (0,81). The HAQ score doesn9t correlate with SLICC (p=0,461) and with SLEDAI (p=0,172), but it was correlated with the neuropsychiatric manifestations. So the psychiatric affectation influences performing the usually daily activities (p=0,005). When assessing Illness Perception Questionnaire, patients considered their life seriously affected by pain, mean value 6,3 (2,6), 72% consider the illness will be present for the rest of their lives, 90% appreciate that treatment is improving the QoL mean 9,3 (1,3), 68% consider to have many severe symptoms, more than 80% patients consider themselves preoccupied for their illness, more than 70% are emotionally affected by their illness and 48% consider stress being the main determinant factor of their illness. Questionnaire EQ-5d: 10% must stay in bed due to the disease, 8,33% can not wash or dress themselves, 12,5% are unable to perform usual daily activities, 62% experience pain or a moderate uncomfortable state and 65% are worried and or depressed. Cardio-vascular manifestations are correlated with diminished mobility (p=0,002) leading to deficiency in self care (p=0,002)also correlated with renal (p=0,034) and psychiatric (p=0,022) manifestations. Psychiatric manifestations also affect usual daily activities (p=0,05). Age is correlated with pain and uncomfortable state (p=0,004) but the responses to this item of EQ5D are not correlated with organ affectation, biological modification or disease activity scores. The presence of cutaneous manifestations is correlated with anxiety and depression (p=0,02). Based on SF36 when calculating the mean value on each category was observed all the 8 categories affected - smallest values at physical role (37,32) and general health (34,21), less influenced part being the social function (51,32).None of this items of SF 36 is correlated with SLICC or SLEDAI. Conclusions SLEDAI as well as SLICC or specific organ involvement give no indications over the patients QoL, which must be assessed by performing HAQ and SF 36. References Lupus. 2016 Mar 16. pii: 0961203316638934. [Epub ahead of print]Gender differences in systemic lupus erythematosus concerning anxiety, depression and quality of life.Macêdo EA1, Appenzeller S2, Costallat LT2. Disclosure of Interest None declared

Recent advances in understanding the multifaceted pathobiology of rheumatoid arthritis have highlighted the pivotal role and continuing crosstalk between activated immune cells, pro-inflammatory cytokines, and matrix-degrading mediators, promoting chronic inflammation as well as irreversible tissue damage within an autoimmune background. B cells are widely recognized as leading players in immune-mediated pathology based on their ability to produce not only different patterns of autoantibodies and driving cytokine synthesis but also as independent antigen-presenting cells and by modulating the specific activation of T cells. Overwhelming evidence emphasized the role of BAFF, a B-cell-activating factor, and BAFF receptors (TACI, BCMA, BAFF-R) in promoting B-cell homeostasis, proliferation, and survival under normal and autoimmune systemic disorders. We systematically reviewed data from literature focusing on BAFF, its homolog molecule APRIL, and BAFF-binding receptors biology, dysregulation of BAFF/BAFF receptor signaling in autoimmune settings, and current status of targeting BAFF/BAFF receptor pathway for rheumatoid arthritis.

Objectives To determine the causes of death and risk factors in systemic sclerosis (SSc). Methods Between 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Candidate prognostic factors were tested by Cox proportional hazards regression model by single variable analysis, followed by a multiple variable model stratified by centres. The bootstrapping technique was used for internal validation. Results We identified 2719 French certificates of deaths related to SSc, mainly from cardiac (31%) and respiratory (18%) causes, and an increase in SSc-specific mortality over time. Over a median follow-up of 2.3 years, 1072 (9.6%) of 11 193 patients from the EUSTAR sample died, from cardiac disease in 27% and respiratory causes in 17%. By multiple variable analysis, a risk score was developed, which accurately predicted the 3-year mortality, with an area under the curve of 0.82. The 3-year survival of patients in the upper quartile was 53%, in contrast with 98% in the first quartile. Conclusion Combining two complementary and detailed databases enabled the collection of an unprecedented 3700 deaths, revealing the major contribution of the cardiopulmonary system to SSc mortality. We also developed a robust score to risk-stratify these patients and estimate their 3-year survival. With the emergence of new therapies, these important observations should help caregivers plan and refine the monitoring and management to prolong these patients' survival.

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

Background Sjogren Syndrome (SS) affects mainly exocrine glands. The latest diagnostic criteria designed for clinical studies are also used as guidance in clinical practice [1]. Ultrasonography (US) demonstrates specificity and sensibility in parotid and submandibular gland evaluation (SG). Parameters considered are echogenicity, homogeneity and margins regularity [1,2,3]. To standardize the assessment of B mode US of SG, different semi-quantitative scores were proposed. Objectives To apply and compare 9 US semi-quatitative scoring systems in B mode scanning of salivary glands in Sjogren Syndrome. Methods A research using keywords “salivary glands”, “ultrasonograpy”, “Sjogren Syndrome”, “semi-quantitative score” in Medline/Pubmed was performed. There was a selection of most relevant articles. There were not considered relevant publications with impact factor <1. We performed the examination on SG in B mode US and applied these scores (De Vita, Niemela, Hocevar, Salaffi, Yukinori, Cornec,Theander) to our patients (primary and secondary SS). Results Eighty four SG in patients diagnosed with primary and secondary (57.15%) SS were assessed. In the group of patients with SSA/SSB presence (85.7%), mean score was De Vita 1.78+/-1.21, Niemela 2.56+/-2.17, Hocevar and Wernicke 2.39+/-2.14, Salaffi 2.83+/-2.52, Yukinori 2.39+/-2.14, Milic 3.39+/-2.14, Cornec 1.78+/-1.215, Theander 1.28+/-0.752. Schirmer test and the need for using the artificial tears was correlated to SG alterations in scoring systems proposed by Niemela (r 0.465, p<0.05) and Salaffi ( r 0.496, p<0.02). All scoring systems were strongly correlated between them (r>0.8, p<0.01). Conclusions Inhomogeneity of parenchyma was considered in all scoring systems. Others considered relevant glandular dimension and margins regularity [2,3.4]. There was no difference between the scoring systems. Xeroftalmia valided through Schirmer test is correlated to SG parenchymal alterations. Our data is an update about semi-quantitative scoring systems in US of SG in SS. References Vitali C, Bombardieri S, Jonsson R et al. Classification criteria for Sjögren9s Syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. AnnRheum Dis 2002;61:554–8. Makula E, Pokorny G, Palkό A.The place of magnetic resonance and ultrasonographic examinations of the parotid gland in the diagnosis and follow-up of primary Sjögren9s syndrome. Rheumatology (Oxford). 2000;39(1):97–104. Niemelä RK, Takalo R, Hakala M. Ultrasonography of salivary glands in primary Sjogren9s syndrome. A comparison with magnetic resonance imaging and magnetic resonance sialography of parotid glands. Rheumatology (Oxford). 2004 Jul;43(7):875–9. El Miedany YM, Ahmed I, El Gafaary M. Quantitative ultrasonography and magnetic resonance imaging of the parotid gland: can they replace the histopathologic studies in patients with Sjogren9s syndrome? Joint Bone Spine.2004;71(1):29–38. Disclosure of Interest None declared

Spondyloarthritis (SpA) patients do not respond uniformly to TNF inhibitor therapy, some of them losing their beneficial evolution and becoming primary or secondary non-responders. Hence, questions have been raised whether this situation is linked to predictive genetic factors, individual characteristics or to disease activity. Studies have incriminated immunogenicity as being responsible for the loss or lack of response. It appears that the development of anti-drug antibodies leads to a decrease in drug levels with further impact on patients’ clinical state. This brief review aims to clarify some of the processes involved in the immunogenic phenomenon for patients with SpA, based on the current published literature.

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

Objective: Vitamin D has pleiotropic effects including immunomodulatory, cardioprotective, and antifibrotic properties and is thus able to modulate the three main links in scleroderma pathogenesis. The aim of the study was to evaluate the level of vitamin D in patients with systemic sclerosis and to analyze the associations between the concentration of vitamin D and the features of systemic sclerosis.Material and Methods: Fifty-one consecutive patients were evaluated for visceral involvement, immunological profile, activity, severity scores, and quality of life. The vitamin D status was evaluated by measuring the 25hydroxy-hydroxyvitamin D serum levels.Results: The mean vitamin D level was 17.06 +/- 9.13 ng/dL. Only 9.8% of the patients had optimal vitamin D levels; 66.66% of them had insufficient 25(OH) D levels, while 23.52% had deficient levels. No correlation was found between vitamin D concentration and age, sex, autoantibody profile, extent of skin involvement, or vitamin D supplementation. Vitamin D levels were correlated with the diffusing capacity of the lung for carbon monoxide (p=0.019, r=0.353), diastolic dysfunction (p=0.033, r=-0.318), digital contractures (p=0.036, r=-0.298), and muscle weakness (p=0.015, r=-0.377) and had a trend for negative correlation with pulmonary hypertension (p=0.053, r=-0.29).Conclusion: Low levels of vitamin D are very common in systemic sclerosis. Poor vitamin status seems to be related with a more aggressive disease with multivisceral and severe organ involvement, especially pulmonary and cardiac involvement.

Background/Objective. Disease activity in spondyloarthritis (SpA) is evaluated through conventional scores such as BASDAI or ASDAS. The aim of this study was to determine disease activity in SpA patients under biological therapy with further assessment on the quality of each activity indicator and their clinical value together with their impact on NSAID intake. Materials and methods. Over nine months (January–August 2015) we enrolled 100 patients with SpA on anti-TNF therapy (33% IFX; 35% ADL; 32% ETA). We collected demographic, clinical (BASDAI, ASDAS, PtGA) and laboratory data (ESR, CRP). Frequency of NSAID ingestion expressed as days per week was included in a questionnaire. Considering their biologic treatment, patients were divided into disease activity groups and sample characteristics were correlated and compared. Statistical analysis was performed with SPSS 20.0, using specific tests such as Pearson, Spearman or ANOVA. Results. Patients with active disease, stated by both activity scores had an increased NSAID intake. Out of 86% of patients with a BASDAI under 4, only 82.6% had an ASDAS-CRP lower than 1.3. BASDAI correlated to both ASDAS scores (r = 0.65 and 0.71, P<0.001) and PtGA correlated to both disease activity scales, with a stronger correlation to BASDAI (r = 0.912, P<0.01) than ASDAS (r = 0.67 and 0.71, P<0.01 for both). Conclusion. We proved that disease activity scores have good discriminatory power, with a strong correlation between the subjective BASDAI and PtGA. However, ASDAS-CRP proves to be more rigorous than other variables when targeting high and low disease activity, thus indicating the need of an objective component in assessing these patients.

AIM Adalimumab effectiveness on clinical, functional and work-related outcomes was evaluated in patients with active ankylosing spondylitis or psoriatic arthritis treated in routine clinical practice in central-eastern Europe. METHODS Patients (n = 555) were followed for 12 months. Primary end point was percentage of patients with a treatment response (≥50% decrease from baseline in Bath Ankylosing Spondylitis Disease Activity Index or ≥1.2 point decrease from baseline in Disease Activity Index-28 joint for axial or peripheral symptoms, respectively). Functional status was evaluated by the Bath Ankylosing Spondylitis Functional Index and Health Assessment Questionnaire Disability Index. Working ability was evaluated by the Work Productivity and Activity Impairment Questionnaire - Specific Health Problem. RESULTS 76.1% of patients with axial symptoms and 83.5% with peripheral symptoms achieved a treatment response. Frequency of extra-articular manifestations decreased. Improvements were observed in functional status and workability. No new safety signals were observed. CONCLUSION Adalimumab was effective and well tolerated during real-world use in central-eastern Europe.

Background Systemic Lupus Erhytematosus (SLE) is a multiorgan disease in which pattern of evolution is linked to activity and damage. When the disease starts at an early age, all the outcomes might be influenced by this. Objectives The main focus of this study is to describe the differences between juvenile and adult onset SLE in a Romanian cohort. Methods 101 SLE patients were evaluated between March 2015 and December 2015. Patients were diagnosed with SLE according to ACR classification criteria and splited in 2 groups, taking into account age at disease onset: before and after 18. All patients agree to participate in this study. Data about demographic, clinical or serological characteristics, activity (SLEDAI) or damage (SLICC damage index SDI), treatment were collected. Statistical analisys was performed with SPSS 20.0. Results Our cohort was made from 18 patients with juvenile onset SLE and 83 with adult onset SLE. Mean age at diagnosis was 14.05 versus 37.89. The juvenile onset SLE group had significant more autoimmune disease as family background, mainly SLE and rheumatoid arthritis (33.3% versus 12.04%, p 0.025), significant more severe organ involvement during the disease evolution: SLE related neurologic involvement 33.3% versus 12% (p 0.025) and renal involvement 50% versus 16.8% (p 0.023), more frequent anti dsDNA Ab positives 88.8% versus 60.24% (p 0.021). This more severe pattern of evolution is also reflected by immunosuppressant therapy, Cyclophosphamide being used in juvenile subgroup in 66% cases versus 35.3% in adults (p 0.014). Interesting, there were no differences between cumulative damage between groups, as measured by SDI (p 0.24), with the mention that in juvenile onset patients, SDI>1 was more frequent SLE related. This is in line with the fact that side effects of the corthicotherapie were identified more in adult onset SLE 61.44% versus 33.3% in juvenile onset (p 0.029). Conclusions Our data clearly show that juvenile onset SLE has more genetic background and it is more prone to a severe disease, renal and neurologic involvement being more frequent than in adult onset form. Severity seems to be related both to the activity, but also to fast damage accrual over time. Controlling the disease activity must be our goal, since cumulative irreversible damage is mainly SLE related for our patients. References Watson, L., Leone, V., Pilkington, C., et all, on behalf of the UK Juvenile-Onset Systemic Lupus Erythematosus Study Group, “Disease activity, severity, and damage in the UK juvenile-onset systemic lupus erythematosus cohort”. Arthritis & Rheumatism, 2012, 64: 2356–2365. doi: 10.1002/art.34410. Disclosure of Interest None declared