Acta reumatologica portuguesa最新文献

Purpose: COVID-19 changed the dynamics of all healthcare system, leading to the restructuring of inpatient teams as well as the emergency department. Scheduled surgeries were suspended, operating rooms were closed, and anesthesiologists redistributed among the various intensive care units. At the Centro Hospitalar do Baixo Vouga the number of patients admitted to the emergency department decreased to approximately 8.000 during the period of lockdown which ranged from 18th March to 1st June 2020. The aim of this study was to compare the number of patients presenting with hip fractures during the first wave of the COVID-19 pandemic with the equivalent period in 2019 and to analyze postoperative outcomes.

Methods: An observational retrospective study was conducted in two different periods. Patients over the age of 50 years admitted with hip fracture were included for analysis. The data was collected from the hospital database. A general descriptive analysis was performed.

Results: There was an overall reduction in the number of admissions due to hip fractures in Period 2020 compared with homologous Period in 2019 (68 patients and 94 patients, respectively). No statistically significant differences could be found regarding age, gender, ASA grade and pre-admission residence among patients admitted during these both periods. Nursing home patients in Period 2020 had a longer hospital stay (p=0.03), independently of the functional status (p=0.07). There were no statistically significant differences in the time it took the patient to go to the emergency department after the fall, place where the fracture had occurred, waiting time to perform the surgery, type of treatment performed, post-surgical complications and mortality. There was no relationship between mortality and the time it took the patient to access the emergency department (p=0,487), or mortality and the mean length of stay in the hospital (p=0,151). All the patients admitted to the emergency department in Period 2020 were negative to PCR test for SARS-CoV-2.

Conclusion: The measures taken by the hospital during the pandemic had no impact in the healthcare provided to the admitted patients. This should be taken into account in order to optimize the efficiency of the health care system in future outbreaks.

Introduction: Long-term survivors of cystic fibrosis (CF) have a dramatic increase in the risk of osteoporosis and incident fracture. The objective of this work is to characterize a CF related bone disease in a Portuguese cohort of CF patients.

Methods: We performed a cross-sectional, observational study on a cohort of CF adult patients. Clinical status, laboratory parametres, nutrition, lung function tests, genetics and bone mineral density (BMD) data were collected from a CF reference centre.

Results: Of 30 patients, 53.3% were males (n=16). Median age was 32.5 (27.0; 32,5) and median body mass index (BMI) was 22,04 (19,85; 24,55), with 4 patients (13.3%) being underweight (BMI<18.5 kg/m²). Four patients (13.3 %) were diagnosed with osteoporosis and 15 patients (50%) has low BMD. Among them, 2 (6.7%) had fragility fractures. A moderate correlation was found between the lumbar spine (LS) BMD and BMI and, as expected, femural neck BMD and LS BMD has moderate to strong correlations with BMD Z scores.

Conclusion: Despite the young middle age we found a high prevalence of low BMD and osteoporosis in patients with CF. Early recognition and treatment are the most effective strategies for reducing the morbidity due to osteoporosis in these patients.

This is the first detailed description of an erosive seronegative polyarthritis in the Vogt-Koyanagi-Harada Syndrome in Portugal.

Objective: Biobanks for research (BBR) have enormous value for research, including those specifically oriented to chronic diseases. Knowing public attitudes and perceptions is key to design and implement patient-centered BBR. We assessed patient awareness, perception and choices among rheumatology outpatients regarding aging biobanking activities.

Methods: We conducted a cross-sectional survey of patients, aged 50 or older, attending an outpatient rheumatology tertiary department. Demographic data and perceptions about biobanking were collected and statistical analysis was performed.

Results: 132 valid questionnaires were obtained (mean age: 63,4; 68,2% female; mean education years: 8,35). 61,7% of respondents did not know the specific term "biobank", 57,7% knew they could donate biological material for BBR, 89,9% agreed with these infrastructures and 88,3% would consider participation Those participants with more years of education were more knowledgeable and prone to biobank participation. Willingness to participate in BBR was mainly related (86,4%) to the advancement of scientific knowledge and not individual gain. Scientific research institutes were indicated as the most adequate institutions to manage BBR. Informed consent, anonymity and confidentiality ranked as top requisites for biobank participation. 61,3% of respondents expressed their agreement with aging biobanks, considering these as a sign of respect for specific problems of people of older ages such as higher disease burdens.

Conclusion: Knowledge of biobanks was found to be limited. Participants were positive toward the setting up of biobanks in general and patient-centered aging biobanks in particular. Knowledge about biobanks and acceptance were higher among participants with higher education years.

Aims: Few reports have focused on the distal interphalangeal (DIP) joint in patients with rheumatoid arthritis (RA). The purposes of this study were to evaluate the frequency of bone erosion of the DIP joint, and to determine the factors associated with its deformity.

Methods: This study reviewed 204 patients with RA in whom radiographs of hands were obtained. According to the presence/absence of bone erosion of the DIP joint, patients were divided into two groups (DIP-positive and DIP-negative groups). Additionally, wrist, metacarpal phalangeal (MP), thumb interphalangeal (IP), and proximal interphalangeal (PIP) joints were evaluated. Clinical variables such as age, sex, body mass index, disease duration, disease activity (DAS28-CRP), and drug use were investigated.

Results: Regarding the radiological findings of the DIP joint, 32 patients (15.7%) were allocated to the DIP-positive group and 172 patients (84.3%) to the DIP-negative group. The mean age, disease duration, DAS28-CRP, and the rate of corticosteroids usage were significantly higher in the DIP-positive than in the DIP-negative group (p = 0.0031, 0.0062, 0.0342, and 0.0011, respectively). Radiologically, concomitant bone erosions of the wrist, MP, thumb IP, and PIP joints were significantly more common in the DIP-positive than in the DIP-negative group (p < 0.01 for all four joints). Multivariate analysis demonstrated that advanced age, long disease duration, and the presence of radiological bone erosion of the PIP joint were independently associated with bone erosion of the DIP joint (p = 0.0480, 0.0307, and 0.0021, respectively). Accordingly, in patients with DIP erosions, mean DAS28-CRP was significantly higher in patients with <5 years (n = 10) than in those with ≥5 years of disease duration (n = 22, p = 0.0088).

Conclusions: Bone erosion can be observed at the DIP joint in patients with RA, and these cases frequently shows bone erosions of other finger joints, such as PIP joint. In addition, bone erosion can be observed soon after the onset of RA caused by uncontrolled disease activity in some patients with RA.

Tumor necrosis factor alpha inhibitors (TNFi) are basilar treatments in a number of inflammatory rheumatic conditions and autoimmune phenomena such as de novo neuroinflammatory events were already described in these populations under TNFi. We conducted a single-center retrospective study in a cohort of rheumatic patients treated with TNFi to characterize neurological demyelinating/inflammatory disease in these patients. We report 3 cases (n= 744): all of them had spondyloarthritis, the onset of neurological manifestations occurred between 37 and 58 years old and all of them initially presented with an optic neuritis. The neurological symptoms emerged between 13 and 26 months after starting TNFi. All patients discontinued treatment with TNFi, but one resumed therapy with symptomatic worsening, having to interrupt treatment again. All patients, latter on, fulfilled multiple sclerosis (MS) McDonald criteria 1 and were diagnosed with relapsing-remitting MS. Our study support the prior view of a risk, disease-dependent or agent-dependent, although a causal relationship is yet to be enlightened.

Objective: The aim of this study was to evaluate the self-reported impact of mandatory confinement occurring in the first wave of the SARS-CoV-2 pandemic in Portuguese patients with rheumatoid arthritis (RA), as a means to improve care during this and in future pandemics.

Material and methods: The web-based survey COVIDRA was developed to assess 5 domains including RA symptoms, attitudes towards medication, employment status, physical exercise and mental health. The questionnaire was sent to RA patients through e-mail and social media of the Portuguese Society of Rheumatology and two patient associations; and it was filled locally at two rheumatology centers in Lisbon. Recruitment took place during June and July 2020.

Results: We obtained 441 valid questionnaires. Most respondents were female (88.4%), caucasian (93.6%) with a mean age of 58 years. The majority had disease lasting >10 years and were treated with csDMARDs (63.2%) and/or bDMARDs/tsDMARDS (23.7%). Over 40% experienced symptom worsening during confinement, almost half considered moderate or severe. Mobility restriction and increased stress, anxiety or depression were responsible for this worsening. Only 2.5% reduced or withheld their immunosuppressive medication due to fear of becoming infected with SARS-CoV-2. After confinement, 16.2% of those previously employed were in a lay-off regime and 3% lost their jobs. Most employed RA patients practiced telework during confinement. The majority of patients decreased or did not practice any physical exercise (80.5%). Symptoms of anxiety and depression developed or worsened in 67.3% and 51.9% respectively, approximately one third were considered moderate or severe.

Conclusions: Portuguese RA patients experienced significant symptom worsening, anxiety and depression during the first wave confinement. Only a minority changed their immunosuppressive treatment for fear of SARS-CoV-2 infection. Published literature on these matters shows results very similar to ours.

Introduction: Generalized joint hypermobility is a clinical feature that is associated with excessive joint laxity, which can occur alone or with various inherited disorders. The term of benign joint hypermobility or joint hypermobility is used when the presence of musculoskeletal symptoms in subjects with generalized joint hypermobility in the absence of demonstrable systemic rheumatic diseases. In recent studies, it was shown that there is a strong relationship between structural and functional gastrointestinal disorders and joint hypermobility. We aimed to analyze the prevalence of celiac disease in a group patient with joint hypermobility.

Patients and methods: The study included the 2 groups of children (i) Group 1; patients with joint hypermobility that were followed in pediatric rheumatology outpatient clinic (n=131). (ii) Group 2; healthy children without known chronic diseases (n=995). Demographic features, clinical findings, accompanying symptoms and anthropometric measurements of all patients were recorded. All cases were screened for celiac disease by serological marker and histopathological examinations if serological marker was positive.

Results: There was no difference between two groups for age, gender, presence of malnutrition and accompanying symptoms (p>0.05). Serology positivity of anti-tissue transglutaminase IgA >20 RU/ml was found in seven patients with joint hypermobility. After histopathological examinations, asymptomatic celiac disease was detected in one (n=1, 0.9%) and potential celiac disease in six patients (n=6, 5.3%). There were six (0.6%) patients with positive serology in the control group. Celiac serology positivity and potential celiac disease were higher in patients with joint hypermobility (6.2%, vs. 0.6%, OR: 10.9, 95% CI: 3.6-33, p < 0.001 and 5.3%, vs. 0.4%, OR: 13.9, 95% CI: 3.6-50, p < 0.001, respectively), but no significant difference was found on the prevalence of asymptomatic celiac disease (0.9%, vs. 0.2%, OR: 4.4, p=0.22).

Conclusion: Our study shows the increased prevalence of potential celiac disease in patients with joint hypermobility. Serological screening of celiac disease is recommended for to rule out organic problems in the presence gastrointestinal symptoms in patients with BJH.

Background: many Juvenile Idiopathic Arthritis (JIA) patients reach inactivity while medicated, but there are no guidelines to determine the moment or method for discontinuing medications. We present the flare rates and remission and possible influencing factors after therapy discontinuation in children with JIA.

Methods: data was collected from charts of JIA patients (n=70) in remission on medication, who had their drugs withdrawn.

Results: Seventy patients fulfilled inclusion criteria and were included for analysis. The mean time of inactive disease on medication until tapering or withdrawal was 15.6±6.7 months; 45 (64.3%) patients remained in remission and 25 (35.7%) flared. There was no difference between groups regarding sex, age, JIA subtype, disease duration, time in remission on medication and scheme of therapy withdrawal. Patients who fulfilled Wallace criteria for remission off medication had lower flare rates than those who did not achieve 12 months of remission after the medication withdrawal (p<0.0001). Patients who used biologic DMARDs plus synthetic DMARDs appeared to flare more (77.8% vs 29.5% respectively, p=0.008) and presented shorter periods of inactivity off medication (15.3±24.7 vs 32.3 ± 31.7 months respectively, p=0.049) compared to those who used only synthetic DMARDs.

Conclusion: It is possible that gradual drug tapering is not necessary for JIA patients, but caution must be exerted in those patients using biologic DMARDs, weighing carefully the decision to withdraw medication, due to their higher flare rates and shorter times of inactive disease after the medication withdrawal.