Background: The vasculitides are a group of rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is a web-based electronic clinical record, created in 2008, which currently includes specific modules for 12 diseases and > 20,000 patients registered from 79 rheumatology centres. On October 2014, a dedicated module for vasculitis was created as part of the European Vasculitis Society collaborative network, enabling prospective collection and central storage of encrypted data from patients with this condition. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, assessment tools, and treatment were collected. We aim to describe the structure of Reuma.pt/vasculitis and characterize the patients registered since its development. Results: A total of 687 patients, with 1945 visits, from 13 centres were registered; mean age was 53.4 ± 19.3 years at last visit and 68.7% were females. The most common diagnoses were Behçet's disease (BD) (42.5%) and giant cell arteritis (GCA) (17.8%). Patients with BD met the International Study Group criteria and the International Criteria for BD in 85.3 and 97.2% of cases, respectively. Within the most common small-and medium-vessel vasculitides registered, median [interquartile range] Birmingham Vasculitis Activity Score (BVAS) at first visit was highest in patients with ANCA-associated vasculitis (AAV) (17.0 [12.0]); there were no differences in the proportion of patients with AAV or polyarteritis nodosa who relapsed (BVAS≥1) or had a major relapse (≥1 major BVAS item) during prospective assessment (p = 1.00, p = 0.479). Biologic treatment was prescribed in 0.8% of patients with GCA, 26.7% of patients with AAV, and 7.6% of patients with BD. There were 34 (4.9%) deaths reported.

BackgroundPatients with rheumatic inflammatory conditions have an increased risk of premature death due to cardiovascular causes. It can be explained by the unfavourable interaction between the inflammatory process and the traditional cardiovascular risk factors. In obesity, especially if visceral, and in rheumatic diseases, there is production of pro-inflammatory cytokines, which contributes to an increase in cardiovascular risk. The influence of body mass index (BMI) on the evolution, activity and quality of life in rheumatoid arthritis (RA) and in psoriatic arthritis (PsA) has been proven. However, studies evaluating the influence of the abdominal circumference (AC) and metabolic syndrome (MS) are meagre.ObjectivesTo assess the influence of BMI, AC and MS, on disease activity and quality of life in RA and PA, using parameters of inflammatory activity (sedimentation rate (SR) and C-reactive protein (CRP), Activity Score (DAS28), Visual Analogue Pain Scale (VAS) and Health Assessment Questionnaire (HAQ) and to compare patients with RA and PA.MethodsA cross-sectional study, including 150 patients with RA, diagnosed according to the ACR/EULAR criteria and 75 patients with PsA (CASPAR criteria). Assessment of weight, height, AC, SR and CRP of all patients, clinical and demographic data collection. The presence of MS was assessed according to WHO definition. Participants completed HAQ and disease activity was measured by DAS28. SPSS was used for the statistical analysis, significance level was 2-sided p<0.050.ResultsAge, duration of illness, schooling and professional class were similar in RA and PsA. In RA there was a predominance of females (78.7%), while in PsA a predominance of males (53.3%). There were no differences between the quality of life (by HAQ), or in the disease activity (by DAS28 or by inflammatory parameters). PsA patients had significantly higher BMI and AC. The number of comorbidities was higher in cases of PsA. Dyslipidaemia and hyperuricemia were significantly more frequent in this group of patients. Independently the underlying pathology (RA or PsA), the number of comorbidities correlated positively with DAS28, with HAQ, CRP and SR.In RA group, there was a positive correlation of both BMI and AC with HAQ, also MS associated the highest HAQ values. Overweight/obesity (BMI≥25kg/m2) were associated with at least one painful joint. Still, the risk of having at least one swollen joint was 3.4 times higher in patients with increased AC (95% CI: 1.08-10.39). There was an association between the BMI and AC and the CRP value. Patients with BMI≥25 kg/m2 and with increased AC had DAS28 values significantly higher. MS was associated with significantly higher SR.In PsA group Patients with MS had higher CRP values, more joint pain and higher disease activity according to DAS28. Patients with BMI≥25kg/m2 also had more painful joints and higher CRP values. None of the patients with normal BMI had swollen joints, however 20.4% of overweight patients had at least one swollen joint. There was no association betwee...

Background 25-hydroxy-vitamin D [25(OH) D] insufficiency/deficiency has been associated with many chronic diseases. There are only a few studies in the literature that have analyzed vitamin D status in juvenile idiopathic arthritis (JIA). Objectives Our purpose was to assess the prevalence and associations of 25(OH) D insufficiency/deficiency in children’s and adults with JIA. Methods We have recruited 40 patients with JIA according to the criteria of the International League of Associations for Rheumatology (ILAR). Age, gender, disease duration, radiological joint destructive changes and medical treatments were collected. Questionnaires were performed to evaluate mean sun exposure time and dietary intake of vitamin D. In all we evaluated the current Disease Activity Score for 28 joints (DAS 28). From November 2011 until January 2012, blood samples were drawn in all patients, and phosphorus, calcium, alkaline phosphatase, ESR, CRP and serum 25(OH) D levels were measured. Serum levels between 20-30 ng/ml were classified as vitamin D insufficiency and levels <20 ng/ml as vitamin D deficiency. None of the patients were receiving vitamin D supplementation at the time of or during the year prior to study entry. In the statistical analysis (chi-Square and student T-test for categorical and continuous variables, respectively), a significant association was considered if p<0.05. Results We have evaluated 31 females and 9 males, with a mean age of 22.3 (4-63 years) and disease duration of 14.6±12.1. Regarding the dietary intake, we found that 32.5% had high intake of vitamin D and 27.5% had normal values, while 40% had insufficient intake. The mean sun exposure time throughout the last year was 77.4 minutes/day. The blood levels of calcium, phosphorus and alkaline phosphatase were normal in all patients. We found low levels of vitamin D in 75% of patients:the prevalence of 25(OH) D insufficiency and deficiency were 47.5% and 27.5%, respectively. JIA patients with less sun exposure and higher ESR had significantly lower vitamin D levels. We also found that patients who had greater joint destruction had lower levels of vitamin D, although not statistically significant (p=0.07). There was no association between vitamin D levels and gender, disease duration, intake of vitamin D, CRP, therapy with corticosteroids, number of DMARDs and biological agents per patient or the current DAS28. Conclusions Our study demonstrated that the prevalence of vitamin D deficiency/insufficiency among JIA patients is very high. Although there is a tendency for patients with more joint destruction to present lower levels of vitamin D, sun exposure and ESR seem to be the most important factors. A limitation of this study was the fact that it was performed during winter, when sun daily availability is lower. Future larger, long-term studies evaluating patients with JIA are needed to further elucidate the association between serum 25(OH)D levels and disease activity. Disclosure of Interest None Declared

Using a 12 joint US assessment, a high proportion of patients with DAS28 < 2.6 were found to have inflammatory US activity, and a significant proportion of patients had evidence of tenosynovitis of the tibialis posterior, which may be difficult to clinically detect. A regular and standardized US assessment of RA patients is therefore warranted to complement clinical evaluation and better define disease activity.

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Background Identifying predictors of response to biological therapies in patients with Ankylosing Spondylitis (AS) is of utmost importance, especially in view of the costs and potential side effects of these agents. Objectives To determine baseline predictive factors of response to biological therapies at 12 weeks in patients with AS in daily clinical practice. Methods Patients with AS under biological therapy and followed in the Rheumatic Diseases Portuguese Register (Reuma.pt) were included in this analysis. Reuma.pt is used as an electronic medical record and assessments are performed by rheumatologists. Patients with information at baseline and 12 weeks of follow-up were included in the analysis (n=197). Univariable logistic regression analysis of baseline predictors of ASDAS (improvement ≥1.1) and BASDAI response (improvement ≥2 units or ≥50%) were performed. Variables with a p-value<0.1 were re-tested in multivariable models. When both ASDAS and CRP (or ASDAS and BASDAI) were significant in the univariable analysis, they were included in separate multivariable models, in order to avoid collinearity-problems. Forward selection was performed until the best-fit model was obtained, taking confounding effects into account. Interactions were tested. Results ASDAS response at 12 weeks was predicted by male gender, higher educational level, lower back pain and higher ASDAS (table). When ASDAS was removed from the baseline predictors, both a younger age and higher CRP were significant predictors of ASDAS response. A BASDAI response was independently predicted by age (<40), gender (male), baseline BASDAI (per unit) or ASDAS (per unit) (depending on which was tested in the model). Table 1 ΔASDAS ≥1.1ΔBASDAI ≥2 or ≥50 OR (95%CI)OR (95% CI)OR (95% CI)OR (95% CI) (n=166)(n=135)(n=174)(n=193) Age at start of first biologic (<40 vs ≥40)**4.04 (1.86; 8.78)3.02 (1.56; 5.84)3.43 (1.82; 6.44) Gender (male vs female)3.01 (1.20; 7.57)**2.39 (1.21; 4.74)2.73 (1.36; 5.45) BMI (kg/m2)******** Educational level (years)1.11 (1.01; 1.21)**§§ Patient’s pain (≥4 vs <4; 0-10)0.27 (0.09; 0.84)**§§ ASDAS3.98 (2.19; 7.21)†1.47 (1.03; 2.10)† BASDAI (0-10)§§§1.20 (1.01; 1.43) CRP (≥5mg/l vs <5mg/l)†9.33 (3.89; 22.35)§§ §Not included in the multivariable model **Not selected during multivariable regression analysis (p≥0.05). †Excluded from the model due to collinearity; the same model is repeated with this variable included. Conclusions CRP is as an important predictor of ASDAS response, but not of BASDAI response. A better response can be expected in male- and younger patients. Baseline disease activity predicts a response at 12 weeks. The ASDAS predicts both ASDAS- and BASDAI response and the BASDAI only predicts the BASDAI response. Disclosure of Interest None Declared

BackgroundVarious nationwide studies have been already published to better understand Mixed Connective Tissue Disease (MCTD) (1,2). However, Portuguese data is not available.ObjectivesTo characterize clinical and immunological features of a Portuguese cohort of patients with MCTD.MethodsRetrospective, multicenter study including adult-onset patients with clinical diagnosis of MCTD and fulfilling at least one of the following classification criteria: Sharp, Kasukawa, Alarcón-Segovia or the Kahn’s criteria. Positivity to other autoantibodies besides anti-U1-RNP were allowed. SPSS was used for statistical analysis and significance level was defined as 2-sided p<.05.ResultsA total of 98 patients were included, with a mean age at diagnosis and disease duration of 40.5±13.7 and 7.0±6.5 years, respectively. Most patients were female (87.8%) and Caucasian (70.4%). Raynaud’s phenomenon (96.9%), arthralgia/arthritis (94.9/74.5%) and puffy fingers (60.2%) were the most common and early manifestations. Gastroesophageal (GE), respiratory and muscular involvement were also prevalent, mostly during the follow up, affecting 30.6%, 34.7% and 43.9% of the patients, respectively. Clinical and immunological characteristics are described in Table 1. Males were older at symptom’s onset (65.0 VS 46.7, p=.035), having more respiratory involvement (OR=4.5, 95% CI 1.3-16.4), and positivity to anti-ACPA (OR=20.0, 95% CI: 3.1-129.4). GE involvement occurred more often in Caucasian patients (OR=3.8; 95% CI: 1.0-14.1), while anemia of chronic diseases (OR=2.7; 95% CI: 1.0-7.2), myositis (OR=3.6; 95% CI: 1.3-9.9) and constitutional symptoms (OR=3.2; 95% CI: 1.2-8.3) were more frequent in Afro-American patients, whose were also younger at disease (34.1 VS 50.6, p=.01). After a median follow-up time of 4 (IQR 8) years, 4 deaths occurred (4.1%), mostly (75%) due to infectious complications.Table 1.Clinical and immunological characteristicsClinical ManifestationsAt presentationFollow-upMucocutaneous systemRaynaud’s phenomenon, n (%)85 (86.7)95 (96.9)Puffy hands, n (%)48 (49.0)59 (60.2)SSc-like, n (%)43 (44.8)59 (60.8)SLE-like, n (%)28 (28.9)35 (35.7)Musculoskeletal systemArthralgia/Arthritis, n (%)/n (%)81 (82.7) / 56 (57.1)93 (94.9) / 73 (74.5)Myositis, n (%)26 (25.6)43 (43.9)Hematological system, n (%)46 (46.9)70 (71.4)Respiratory system, n (%)14 (14.3)34 (34.7)Cardiovascular system3 (3.1)4 (4.1)Pulmonary hypertension*2 (2.0)15 (15.3)Gastroesophageal involvement, n (%)11 (11.2)30 (30.6)Renal involvement, n (%)2 (2.0)10 (10.2)Neurological involvement, n (%)6 (6.3)14 (14.3)Constitutional symptoms, n (%)26 (26.5)30 (30.6)Immunological characteristicsAnti-dsDNA, n (%)21 (21.4)Anti-smith antibody, n (%)21 (21.4)Anti-Ro/SSA, n (%)31 (31.6)Anti-La/SSB, n (%)7 (7.1)Anti-centromere, n (%)3 (4.1)Rheumatoid Factor, n (%)39 (39.8)Anti- anti-citrullinated protein antibodies, n (%)6 (6.1)Antiphospholipid antibodies, n (%)7 (7.1)Myositis antibodies, n (%)9 (9.2)Complement activation, n (%)27 (27.6)Hypergammaglobulinemia, n (%)51 (52.0)Legend: Anti-dsDNA: anti-double stranded deoxyribonucleic acid antibody; SLE: systemic lupus erythematosus, SSc: systemic sclerosis. *No information regarding cardiac catheterism, then compatible alterations in the echocardiogram.ConclusionRaynaud’s phenomenon, puffy fingers and arthritis were the most common manifestations in Portuguese patients, with similar proportions found in literature (1,2). However, we reported some differences in mucocutaneous, renal and serosa involvement and higher prevalence of probable pulmonary hypertension (1,2), which may be explained by the heterogeneity of the inclusion criteria. Except for respiratory, myositis, GE and constitutional symptoms, there were no differences regarding gender and ethnicity.Here, we characterize the largest cohort of MCTD in Portugal.References[1]Cappelli S, et al. Semin Arthritis Rheum. 2012 Feb;41(4):589–98.[2]Alves MR et al. Clin Exp Med. 2020 May;20(2):159–66.Disclosure of InterestsNone declared

BackgroundBehcet's disease (BD) is a chronic and multisystemic inflammatory disorder affecting the skin, mucosa, joints, eyes, arteries, veins and the nervous and gastrointestinal systems. The symptoms may be separated by long or short intervals, occur simultaneously or in sequence, and exhibit a pattern of exacerbation and remission. The disease itself or the impact of the symptoms affect patients physically, mentally and socially with a negative effect in the quality of life (QoL).ObjectivesTo determine the health status and quality of life in patients with BD followed in a rheumatology unit and to identify associated demographic and disease-related parameters influencing them.MethodsCross-sectional study of 44 BD patients and 39 healthy controls matched for age and sex. All subjects completed the Health Assessment Questionnaire (HAQ) to assess impairment in daily activities due to illness, Short Form-36 (SF-36) and EuroQol Visual Analogic Scale (EQ-VAS) to assess health related quality of life (HRQL). The disease characteristics, including disease duration and clinical involvements were collected. The Birmingham Vasculitis Activity Score (BVAS) was applied for the evaluation of current disease activity among BD patients. P value <0.05 was defined as statistically significant.ResultsAmong 44 patients, 35 (79.5%) were female, with a mean age of 40.07 years and mean disease duration of 5.93 years. BD patients had significantly higher HAQ score (p=0.009) and lower levels of SF-36 (p<0.001) than the healthy controls. The predominant contributors to this low SF-36 were general health, vitality and role-emotional domains. In comparison with healthy controls, patients with inactive disease (BVAS=0) also had a higher HAQ, but without significant differences (p=0.53). The total SF-36 score also showed lower levels in patients with inactive disease (p=0.04), but when compared the different components, only half of them maintained significant differences, namely role-physical (p=0.03), general health (p<0.001), vitality (p<0,001) and social functioning (p=0.05). The controls showed a higher EQ-VAS, with a mean of 88.44 comparing to 68.36 in all BD patients (p<0.001) and 74.12 in the subgroup with inactive disease (p=0.003). SF-36 score was negatively correlated with HAQ (r=-,553, p<0.001) and positively correlated with EQ-VAS (r=.388, p<0.001). Longer disease duration correlated with lower levels of only some SF-36 domains, namely physical functioning (r=-.436, p=0.003), role-physical (r=-.533, p<0.001) and role-emotional (r=-.465, p=0.001). There was no correlation between disease activity and disease duration (p=0.678) or different scores evaluating QoL (p=0.876). The gender was not associated with statistical differences when compared clinical involvements, disease duration, current disease activity, HAQ, EQ-VAS or SF-36 scores. The heterogeneous nature of the disease expression did not allow the study of the association with the levels of health status and QoL.ConclusionsOur findings showed lower levels of QoL and glob...