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...
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
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