ARP Rheumatology最新文献

Objective: To perform a systematic literature review (SLR) aimed at evaluating the efficacy and safety of pharmacological and non-pharmacological treatments for Raynaud's phenomenon (RP) and digital ulcers (DU) in patients with systemic sclerosis (SSc) and other connective tissue diseases (CTD), in order to inform the Portuguese recommendations for managing RP and DU in these patients.

Methods: A SLR was conducted until May 2022 to identify studies assessing the efficacy and safety of pharmacological and non-pharmacological interventions for RP and DU in SSc and other CTD. Eligible study designs included randomized controlled trials (RCTs), controlled clinical trials, and their extensions for assessing efficacy and safety of interventions. Observational studies with a comparator were included for evaluating the efficacy and safety of non-pharmacological interventions and safety of pharmacological interventions. The risk of bias of each study was assessed using standard tools.

Results: Out of 71 publications meeting the inclusion criteria, 59 evaluated pharmacological and 12 non-pharmacological interventions. We found moderate quality evidence supporting the efficacy of calcium channel blockers, phosphodiesterase-5 inhibitors, and intravenous prostacyclin analogues in reducing RP frequency, severity, and duration. Intravenous iloprost had a small to moderate effect size in improving DU healing. Phosphodiesterase-5 inhibitors were effective in reducing total DU count, new DU occurrence, and enhancing DU healing. Bosentan effectively prevented new DU in SSc patients. No new safety concerns were associated with these treatments. The studies on non-pharmacological interventions were, in general, of low quality, and had a small sample size. Warming measures decreased frequency and duration of RP attacks; laser therapy improved RP-related outcomes; local oxygen-ozone therapy improved RP outcomes as an add-on therapy; bone marrow mononuclear cell implantation improved DU-associated pain; periarterial sympathectomy and vascular bypass reduced DU number and finger amputation risk.

Conclusion: The available evidence supports the efficacy and safety of pharmacological interventions, namely nifedipine, sildenafil, iloprost, and bosentan in treating RP and DU in patients with SSc and other CTD. Scarce and low-quality evidence does support the use of some non-pharmacological interventions but with only a modest effect size. This SLR underscores the limited availability of high-quality evidence for determining the optimal treatment.

Hypophosphatemia may cause serious complications. Depending on its severity and duration, signs and symptoms range from fatigue to life-threatening events, like severe rhabdomyolysis and mental status changes. Long-term consequences include osteomalacia. Hypophosphatemia may be secondary to the use of parental iron, mostly associated with ferric carboxymaltose (FCM), with an incidence of around 45% to 70%. We describe three cases of hypophosphatemia in patients with chronic iron deficiency anemia, requiring repeated FCM infusions. The patients' presentation to the Rheumatology department included musculoskeletal symptoms of severe hypophosphatemia and long-term hypophosphatemic osteomalacia, with fractures. We aim to raise awareness for ferric carboxymaltose-induced hypophosphatemia, an entity increasingly described in the literature that can be responsible for severe disability or potentially life-threatening adverse events.

In inflammatory rheumatic diseases, including, systemic sclerosis (SSc) there is growing evidence that treatment strategies should not only target disease control in terms of clinical features and laboratory tests but consider distinct interventions to mitigate all domains of perceived disease impact. The results of a multicentric work based on data from the Rheumatic Diseases Portuguese Registry (Reuma.pt)/Scleroderma indicated that the optimization of outcomes for patients with SSc would in all probability require assessment of the needs of individual patients and consider adjunctive interventions in clinical practice to mitigate all significantly affected domains of disease impact. Recently, in June 2023, a task force under the auspices of EULAR, comprising rheumatologists, health professionals and patient advocates published four overarching principles and twelve recommendations for the non-pharmacological management of people living with SSc and systemic lupus erythematosus (SLE).

Axial spondyloarthritis, also referred to as ankylosing spondylitis, is a chronic inflammatory condition that predominantly affects the axial spine but may also present with peripheral arthritis. It falls within the umbrella of disorders known as spondyloarthropathies. In addition to axial spondyloarthritis, this group includes psoriatic arthritis, enteropathic arthritis, reactive arthritis, and undifferentiated spondyloarthropathy, with axial spondyloarthritis being one of the most common. The overall mechanisms underlying the development of axial spondyloarthritis are complex and multifactorial. There is a significant and well-recognized association between axial spondyloarthritis and the HLA-B27 gene, but there have also been non-HLA genes identified in the disease process, as well as certain inflammatory cytokines that play a role in the inflammatory process, such as tumor necrosis factor (TNF). More recently, there has been research and new evidence linking changes in the gut microbiota to the disease process of axial spondyloarthritis. Research into the role of the gut microbiota and gut dysbiosis is a large, ever-growing field. It has been associated with a multitude of conditions, including axial spondyloarthritis. This mini-review highlights the symbiotic relationship of the gut microbiota with the pathogenesis, therapeutic agents and future treatments of axial spondyloarthritis.

Eosinophilic vasculitis is a rare condition due to vascular damage and infiltration of eosinophils into tissues. Antineutrophil cytoplasmic antibody (ANCA), like perinuclear ANCA (p-ANCA), is common in the context of systemic vasculitis, but its association with illicit drug use is unusual. This case report showcases a distinctive scenario featuring a positive pANCA, prompting a meticulous examination of the interplay between vasculitic manifestations and drug abuse.

Dermatomyositis (DM) is an immune-mediated myopathy characterized by proximal skeletal muscle weakness, muscle inflammation, and distinct skin manifestations. Calcinosis cutis, involving the deposition of insoluble calcium salts in the soft tissues, affects approximately 8% of DM patients1 and poses significant treatment challenges. It can complicate with inflammation, ulceration, pain, and local and systemic infections, resulting in considerable morbidity. We present the case of a 69-year-old-woman with dermatomyositis who developed an exuberant form of calcinosis cutis.

Introduction: It is important to assess the risk of vertebral fractures (VFs) in patients with rheumatoid arthritis (RA), as RA is associated with a high risk of VFs. However, the epidemiology and risk of VFs in patients with RA remain inconclusive. The present study therefore clarified the prevalence and associated factors of VFs in patients with RA.

Methods: We included 107 patients (19 men and 88 women) and retrospectively investigated the number and location of VFs, bone mineral density (BMD), RA disease activity score for 28 joints based on C-reactive protein (DAS28-CRP), and history of medication for RA and osteoporosis. Based on the investigated items, we assessed the prevalence of VFs in patients with RA and the association between the clinical parameters of RA patients and VFs.

Results: The average age, disease duration, and DAS28-CRP were 67.9 years old, 14.9 years, and 2.2, respectively. We found that the prevalence of VFs in patients with RA was 30.8%, and 84.8% of patients with VFs and 62.2% of those without VFs had been treated for osteoporosis. We further found that the prevalence of VFs in patients with RA with a history of anti-osteoporotic agent use was 37.8%. In univariate analyses, patients with RA with VFs had significantly higher DAS28-CRP values, a higher rate of corticosteroid use, and lower BMD (p = 0.018, p = 0.004, and p < 0.001, respectively) than those without VFs. A multivariable　logistic regression analysis and ordinal logistic analysis revealed that the DAS28-CRP and BMD were independent factors associated with the presence (p = 0.042 and p = 0.011, respectively) and number (p = 0.036 and p = 0.048, respectively) of VFs.

Conclusions: The prevalence of VFs was relatively high in patients with RA, regardless of the use of anti-osteoporotic agents. A high disease activity score and low BMD are associated with the presence and number of VFs in patients with RA. Based on these findings, to reduce VFs in RA patients, it is important to tightly control the disease activity of RA in addition to osteoporosis treatment.

Objectives: This study aimed to estimate the prevalence of anxiety and depression symptoms and explore the association between these symptoms and clinical and pain characteristics in patients with chronic pain (CP) due to hip and knee osteoarthritis (OA).

Methods: In this cross-sectional study, adult patients with CP and knee and/or hip OA were included. Anxiety and depression symptoms were assessed using the Hospital Anxiety and Depression Scale. Visual analogue scale, Western Ontario and McMaster Universities Arthritis Index (WOMAC) and PainDetect Questionnaire assessed pain characteristics and Health Assessment Questionnaire (HAQ) evaluated functional disability. Correlation coefficients were used to explore the associations between anxiety and depression symptoms and clinical and pain characteristics.

Results: A total of 61 patients (age 66.2±9.4 years, 67.2% female) were included. Most patients (70.5%) had clinically significant anxiety and/or depression symptoms. Patients with anxiety and/or depression symptoms had higher pain severity (p=0.032) and disability (p=0.014). Depression symptoms had a moderate positive correlation with WOMAC physical function subscale (r=0.520), WOMAC total (r=0.511) and HAQ (r=0.405).

Conclusions: Anxiety and depression symptoms are prevalent in knee or hip OA patients with CP and were associated with higher pain severity and functional disability. These findings support the screening of anxiety and depression symptoms in OA patients, in order to develop more effective multidisciplinary treatments.

Objective: In this study, we aimed to assess the transition readiness levels amongst patients with childhood-onset rheumatic diseases. Additionally, we sought to identify and analyze predictive factors associated with better transition readiness skills in adolescent and young adult (AYAs) patients.

Methods: This is a monocentric cross-sectional study that includes patients between 14 and 26 years of age who attended outpatient pediatric and young adult rheumatology appointments between October and December of 2023 and that were diagnosed with an immune-mediated rheumatic disease before reaching 18 years of age, with at least 1 year of disease duration. Patients were presented with a questionnaire that contained demographic and clinical questions, TRACS (Questionário de Preparação da Transição para a Autonomia nos Cuidados de Saúde) questionnaire - a validated Portuguese version of the Transition Readiness Assessment Questionnaire (TRAQ), and Hospital Anxiety and Depression Scale (HADS) questionnaire. Data was analyzed to assess the significant associations between the different variables and transition readiness outcome measured by the TRACS. Descriptive statistics, statistical comparisons and logistic regression analysis were performed.

Results: A total of 69 patients with a median age of 20 [17.5-22.5] were included in this study. The median TRACS score was 4.41 [4.09-4.74]. Significantly higher TRACS scores were observed in patients who were female, 18 years of age or older, had a higher level of education, were employed, had active disease or that belonged to middle-class (when compared to patients belonging to upper- middle class). The logistic regression analysis demonstrated that being a female or having an educational status equal to 12th grade or superior emerged as predictors of higher transition readiness levels.

Conclusions: Our study identified female sex and higher level of education as predictors of increased transition readiness levels. Therefore, healthcare providers should consider these variables when assessing patients for transition readiness and focus on improving transition process, especially in male and less educated AYAs.