Acta reumatologica portuguesa最新文献

Background: Patients with rheumatoid arthritis (RA) report significant levels of disease impact, which are improved, but not fully abrogated by immunosuppressive therapy, even when remission is achieved. This imposes the need for adjuvant interventions targeting the uncontrolled domains of disease impact. Non-pharmacological interventions are widely used for this purpose, but they have not been the object of professional recommendations or guidelines.

Objective: To propose multidisciplinary recommendations to inform clinical care providers regarding the employment of non-pharmacological and non-surgical interventions in the management of patients with RA.

Methods: The EULAR standardized operating procedures for the development of recommendations were followed. First, a systematic literature review was performed. Then, a multidisciplinary Technical Expert Panel (TEP) met to develop and discuss the recommendations and research agenda. For each developed recommendation i) the level of evidence and grade of recommendation were determined, and ii) the level of agreement among TEP members was set. A recommendation was adopted if approved by ≥75% of the TEP members, and the level of agreement was considered high when ≥8. All relevant national societies were included in this construction process to attain their endorsement.

Results: Based on evidence and expert opinion, the TEP developed and agreed on five overarching principles and 12 recommendations for non-pharmacological and non-surgical interventions in patients with RA. The mean level of agreement between the TEP members ranged between 8.5 and 9.9. The recommendations include a broad spectrum of intervention areas, such as exercise, hydrokinesiotherapy, psychological interventions, orthoses, education, general management of comorbidities, among others; and they set the requirements for their application.

Conclusions: These recommendations are based on the consensus judgment of clinical experts from a wide range of disciplines and patients' representatives from Portugal. Given the evidence for effectiveness, feasibility and safety, non-pharmacological and non-surgical interventions should be an integral part of standard care for people with RA. It is hoped that these recommendations should be widely implemented in clinical practice. The target audience for these recommendations includes all health professionals involved in the care of patients with RA. The target patient population includes adult Portuguese people with RA.

Objectives: Self-efficacy is an important factor in the acquisition of self-management skills in patients with chronic diseases. The present study provides a translation and cultural adaptation for the Portuguese population, as well as psychometric properties, of the Self-Efficacy for Managing Chronic Disease 6-Item Scale.

Materials and methods: This is a cross-sectional study. As a first stage, a translation and cultural adaptation were conducted. After preparation, a final version was applied initially to a sample of 30 participants with chronic disease in two phases, with a one-week gap between phases, to assess teste retest reliability. Subsequently, a sample of 217 participants with chronic disease, mean age 42.8 (10.7) years, participated in the study. Participants were supposed to be over the age of 18 and with at least one clinically diagnosed chronic disease. The questionnaire was applied electronically.

Results: The results showed a good test retest reliability (ICC of 0.83, 95% CI: 0.65 - 0.92). Internal consistency met the criterion for a reliable measure (global Cronbach's alpha of 0.95). Item-total correlations of all items were above 0.30. A correlation matrix was considered favorable (KMO = 0.90; Bartlett's sphericity test = 1399.090, p < 0.01). The results confirmed the permanence of the 6 items, as in the original scale.

Conclusions: A Self-Efficacy for Managing Chronic Disease 6-Item Scale is a reliable and valid instrument to assess the patients' self-efficacy for managing chronic diseases in Portuguese, enabling its use in clinical practice and in future studies.

Localized scleroderma (LoS) is a rare condition featuring skin and underlying tissue sclerosis not usually compromising other systems. A subtype of LoS including lesions in the head is further classified as linear scleroderma en coup de sabre (LSeCS). Neurological involvement in LSeCS can reach up to 4% and may include seizures. Cutaneous lesions usually emerge before neurologic symptoms and these oftentimes manifest with intracranial abnormalities. We describe a case of an 11-year-old boy with an onset of self-limited unexplained seizures at 20-months of life. During the first year of follow-up, a midline frontoparietal lesion with alopecia and hypopigmentation was noted and a referral to dermatology and pediatric rheumatology consultation was made. A diagnosis of LEsCS was made. A 10-year follow-up of this patient is presented with favorable outcome. LSeCS is a rare form of LoS most frequently diagnosed in children and adolescents. A meticulous examination of these patients should be performed with particular attention to the face and scalp. The mainstay therapeutical approach is based on methotrexate and corticosteroids. Neurologic abnormalities associated with skin lesions on the head should should raise clinical suspicion of LSeCS.

Objective: To investigate the relationship between body mass index (BMI) and disease activity in patients with Juvenile Idiopathic Arthritis (JIA).

Methods: Patients with JIA, aged ≤18 years, registered at the Rheumatic Diseases Portuguese Register (Reuma.pt) in Portugal and Brazil were included. Age- and sex-specific BMI percentiles were calculated based on WHO growth standard charts and categorized into underweight (P <3), normal weight (3≤P≤85), overweight (85

97). Disease activity was assessed by Juvenile Arthritis Disease Activity Score (JADAS-27). Uni- and multivariate analyses were performed.

Results: A total of 275 patients were included. The prevalence of underweight, normal weight, overweight and obesity was 6.9%, 67.3%, 15.3% and 10.5%, respectively. Underweight patients had significantly higher number of active joints (p <0.001), patient's/parent's global assessment of disease activity (PGA) (p=0.020), physician's global assessment of disease activity (PhGA) (p <0.001), erythrocyte sedimentation rate (ESR) (p=0.032) and overall higher JADAS-27 (p <0.001), compared to patients with normal weight, overweight and obesity. In the multivariate regression, underweight persisted significantly associated with higher disease activity, compared to normal weight (B=-9.430, p <0.001), overweight (B=-9.295, p=0.001) and obesity (B=-9.120, p=0.001), when adjusted for age, gender, country, ethnicity, JIA category and therapies used. The diagnosis of RF- (B=3.653, p=0.006) or RF+ polyarticular JIA (B=5.287, p=0.024), the absence of DMARD therapy (B=5.542, p <0.001) and the use of oral GC (B=4.984, p=0.002) were also associated with higher JADAS-27.

Conclusion: We found an independent association between underweight and higher disease activity in patients with JIA. Further studies are needed to understand the underlying mechanisms of this association.

Introduction/objectives: Thiols are crucial anti-oxidant agents that contain a sulfhydryl group; they play an important role in defence against reactive oxygen species. We aimed to determine the thiol/disulphide homeostasis in rheumatoid arthritis (RA) patients in conjunction with its association with disease activity, preclinical atherosclerosis, and other disease-related indices.

Methods: We enrolled 64 RA patients without known cardiovascular (CV) disease or risk factors and 46 healthy controls. Disease activity was evaluated using the Disease Activity Score 28-erythrocyte sedimentation rate (DAS28-ESR). Thiol/disulphide homeostasis was evaluated using a novel automated method, and serum native thiol (NT), total thiol (TT), and disulphide(SS) levels were recorded. The carotid intima media thickness (CIMT) was measured using carotid ultrasound to evaluate preclinical atherosclerosis.

Results: The NT and TT levels were significantly lower in RA patients than in controls (231.7 ± 52.3 vs. 293.6 ± 74.8 µmol/L, p < 0.001; 271.6 ± 52.1 vs. 331.3 ± 68.2 µmol/L, p < 0.001, respectively). There was no difference in SS levels between both groups. The CIMT was significantly higher in RA patients than in controls (0.80 vs. 0.56 mm, p ˂ 0.001). NT levels showed a significant negative correlation withCIMT in patients with RA (r = - 0.253, p = 0.040). In RA patients, NT and TT levels were significantly correlated with ESR (r = - 0.394, r = -0.399), high-sensitivity C-reactive protein (r = -0.413, r = - 0.342), DAS28-ESR (r = - 0.279, r = - 0.312), fibrinogen level (r = - 0.302, r = - 0.346), and anti-cyclic citrullinated peptide titres (r = - 0.305, r = 0.322) (, respectively). The association of thiol levels with CIMT did not arrive at a statistically significant level in multivariable linear regression analysis.

Conclusions: RA patients without known CV disease or risk factors exhibited increased CIMT values and decreased thiol levels; moreover, thiol levels were found to be correlated with disease activity. Further studies are needed to detect the value of thiol/disulphide homeostasis for CV risk stratification and risk prediction in RA patients.

Ocular involvement in spondyloarthritis (SpA) is a frequent extra-articular manifestation and contributes to the burden of disease. Factors associated with spondyloarthritis-related uveitis (SpA-U) are poorly defined in literature. The influence of smoking status on the occurrence of uveitis in SpA is controversial. To clarify the factors associated with SpA-U, we performed an observational cross-sectional study in a Tertiary Rheumatology Centre. Factors independently associated with uveitis were determined by logistic regression models. The study included 164 patients fulfilling the ASAS criteria for axial SpA with follow-up visit between January and June 2019. Smoking was independently associated with uveitis (OR=2.54; 95%CI [1.01-6.42]; p=0.03). Our study emphasizes the importance of smoking cessation in SpA which may have a positive effect in different disease features like uveitis and overall prognosis.

Background: Remission/ low disease activity (LDA) are the main treatment goals in rheumatoid arthritis (RA) patients. Two tools showing the ability to predict golimumab treatment outcomes in patients with RA were published.

Objectives: To estimate the real-world accuracy of two quantitative tools created to predict RA remission and low disease activity.

Methods: Multicenter, observational study, using data from the Rheumatic Diseases Portuguese Register (Reuma.pt), including biologic naïve RA patients who started an anti-TNF as first-line biologic and with at least 6 months of follow-up. The accuracy of two matrices tools was assessed by likelihood-ratios (LR), sensitivity (SN), specificity (SP), positive predictive value (PPV), negative predictive value (NPV) and area under the ROC curve (AUC).

Results: 674 RA patients under first-line anti-TNF (266 etanercept, 186 infliximab, 131 adalimumab, 85 golimumab, 6 certolizumab pegol) were included. The median (IQR) age was 53.4 (44.7-61.1) years and the median disease duration was 7.7 (3.7-14.6) years. The majority were female (72%). Most patients were RF and/or ACPA positive (75.5%) and had erosive disease (54.9%); 58.6% had comorbidities. At 6-months, 157 (23.3%) patients achieved remission (DAS28 ESR < 2.6) and 269 (39.9%) LDA (DAS28 ESR ≤ 3.2). Area under the curve for remission in this real-world sample was 0.756 [IC 95% (0.713-0.799)] and for LDA was 0.724 [IC 95% (0.686 -0.763)]. The highest LR (8.23) for remission state was obtained at a cut-off ≥ 67%, with high specificity (SP) (99.6%) but low sensitivity (SN) (3.2%). A better balance of SN and SP (65.6% and 73.9%, respectively) was observed for a cut-off >30%, with a LR of 2.51, PPV of 43.3% and NPV of 87.6%.

Conclusion: In this population, the accuracy of the prediction tool was good for remission and LDA. Our results corroborate the idea that these matrix tools could be helpful to select patients for anti-TNF therapy.

Objective: Systemic sclerosis (SSc) is a complex disorder of unknown etiology. The purpose of this study was to evaluate fibroblast growth factor-23 (FGF-23) serum levels in women with SSc compared with healthy controls and to examine a possible association between FGF-23 serum levels with the presence of calcinosis in SSc patients.

Methods: This cross-sectional study was performed in San Cecilio Hospital, Granada (Spain) from November 2017 to May 2019. Sixty-two women with SSc and 62 age and sex matched healthy controls were included in this study. FGF-23 serum concentration was evaluated by indirect enzyme-linked immunosorbent assay (ELISA).

Results: There was no significant difference in FGF-23 levels between SSc patients and healthy controls (78.2 ± 60.5 vs. 80.3 ± 56.3 pg/mL, p= 0.662). Regarding the characteristics of the disease, we found a relationship between the values of FGF-23 and the presence of calcinosis. The levels of FGF-23 are higher in patients suffering from calcinosis (p= 0.028).

Conclusion: We observed the presence of higher levels of serum FGF-23 in SSc female patients with calcinosis. Therefore, FGF-23 could be a possible therapeutic target for future treatments.