ARP Rheumatology最新文献

Introduction: Patients being on immunosuppressive treatment of any reason, along with other risk factors such as smoking and obesity, are vulnerable to be infected from SARS-CoV2. Aim of this report is to describe a case of a female patient under Rituximab therapy who experienced episodes of lung infection due to Severe Acute Coronavirus 2 (SARS-CoV-2 ) invasion although fully vaccinated.

Case report: A 50-year-old woman, with a past medical history of lupus nephritis on rituximab was diagnosed with lung infection due to SARS-CoV-2. Eight months later, following her last infusion of Rituximab (RTX), she developed moderate Coronavirus Disease 2019 (COVID-19). After a partial recovery, she exhibited exacerbation of respiratory symptoms leading to readmission and invasive oxygenation. She was eventually discharged home after 31 days. Her monthly neurological evaluation did not reveal evidence of disease activity. She later received intravenous immunoglobulin and a decision was made to restart rituximab.

Conclusions: This case raises the possibility of persistent virus shedding and reactivation of severe acute respiratory syndrome coronavirus in a patient with SLE and Rituximab therapy. We emphasize a precise consideration of management of patients with autoimmune disorders during the COVID-19 pandemic.

Transient osteoporosis of the hip (TOH) is an important but often neglected cause of hip pain, which can gradually lead to debilitating mobility and carries risks such as fracture or avascular necrosis. A 39-year-old woman presented to the Rheumatology department two weeks post-cesarean delivery, reporting the onset of left mechanical hip pain since the 33rd week of pregnancy. After delivery, similar complaints emerged on the right side. Hip X-ray showed a decrease in bone density in the left hip. Later, Magnetic Resonance Imaging revealed bilateral bone marrow edema in both proximal femurs. The diagnosis of TOH was established, and the patient was treated with conservative measures. Seven months later, she was asymptomatic. Pregnancy is a recognized risk factor for TOH, especially in the last trimester. It is an important differential diagnosis to consider in cases of hip pain in pregnant or newly breastfeeding women.

Objective: To develop evidence-based recommendations for the non-pharmacological and pharmacological management of Raynaud's phenomenon (RP) and digital ulcers (DUs) in patients with systemic sclerosis and other immune-mediated connective tissue diseases (CTDs).

Methods: A task force comprising 21 rheumatologists, two surgeons (vascular and plastic), two nurses, and one patient representative was established. Following a systematic literature review performed to inform the recommendations, statements were formulated and discussed during two meetings (one online and one in-person). Levels of evidence, grades of recommendation (GoR), and level of agreement (LoA) were determined.

Results: Five overarching principles and 13 recommendations were developed. GoR ranged from A to D. The mean ± standard difference (SD) LoA with the overarching principles and recommendations ranged from 7.8±2.1 to 9.8±0.4. Briefly, the management of RP and DUs in patients with CTDs should be coordinated by a multidisciplinary team and based on shared decisions with patients. Nifedipine should be used as first-line therapy for RP and/or DUs. Sildenafil, tadalafil, and/or iloprost IV are second-line options for severe and/or refractory patients with RP and/or DUs. Sildenafil, tadalafil and/or Iloprost IV, should be prescribed for healing and prevention (also including bosentan) of DUs. In patients with RP and/or DUs, non-pharmacological interventions might be considered as add-ons, but there is limited quality and quantity of scientific evidence supporting their use.

Conclusions: These recommendations will inform rheumatologists, specialist nurses, other healthcare professionals, and patients about a comprehensive and personalized management of RP and DUs. A research agenda was developed to address unmet needs, particularly for non-pharmacologic interventions.

Objectives: To compare spinal and lower limb pain in adolescents regarding prevalence, characteristics, causes, and impact.

Methods: A descriptive cross-sectional study was conducted in 13-year-old adolescents (female n=2210; male n=2353) from the Portuguese Generation XXI birth cohort. Data were collected between 2018 and 2020 through personal interviews by applying the Luebeck Pain Questionnaire. The pain features examined in each anatomical location (back and lower limb) were recurrence, duration, frequency, intensity, perceived causes, and impact on school and leisure activities. Frequencies and the Chi-square test were used.

Results: Questionnaires from 4563 adolescents were analysed, 57.9% had pain in the last three months (main pain in the spine: 11.6%; main pain in the lower limb: 29.0%). Of those, 69.4% and 62.4% reported recurrent pain in the spine and lower limb, respectively. Recurrent pain was more frequent in girls than in boys (spine: 80.0%; 57.0%; lower limb: 70.4%; 58.1% respectively). Pain lasted more than three months in most adolescents (spine: about 60%; lower limb: above 50%); frequency was similarly high in both regions and both sexes (girls: 47.0%; boys: 45.7% in the spine; girls: 45.7%; boys: 40.3% in the lower limb); intensity was rated as high by girls (spine: 45.5%; lower limb: 47.3%) and moderate by boys (spine: 42.0%; lower limb: 41.0%). The leading causes of pain were daily living activities, both for the spine (girls: 65.9%; boys: 76.5%) and the lower limb (girls: 62.2%; boys: 72.1%). Psychosocial causes were the second most common cause of spinal pain (girls: 25.0%; boys: 21.0%). Other causes of lower limb pain were traumatic (girls: 25.5%; boys: 16.6%) and physical factors (girls: 20.7%; boys: 23.8%). Absences from school (girls: 11.7%; boys: 4.8%) and restrictions of leisure activities (girls: 20.7%; boys: 25.2%) were more related to pain in the lower limb.

Conclusion: More than half of the adolescents reported spinal or lower limb recurrent pain, which presents a higher frequency, higher intensity, and longer duration in the spine. However, lower limb pain led to more concurrent limitations.

Adhesive capsulitis, commonly known as frozen shoulder, is a painful and restrictive condition that significantly impairs the quality of life for affected individuals. We present the successful endovascular treatment of adhesive capsulitis in the first-ever case performed in Portugal. A 6-month follow-up with objective results is presented, using the visual analog scale for pain and the Quick Dash questionnaire. Trans-arterial, musculoskeletal embolization, as a cutting-edge technique, offers a promising avenue for patients with adhesive capsulitis unresponsive to traditional management, opening new possibilities for improved outcomes and enhanced quality of life.

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is an emerging adult-onset systemic autoinflammatory disorder affecting multiple organ systems. While lung involvement is common in this syndrome, literature regarding specific patterns is sparse. In this report, we present a case description of a patient with VEXAS syndrome who presented at the emergency department on two separate occasions with acute interstitial pneumonia (AIP) and diffuse alveolar hemorrhage (DAH). A literature review with a comparison of our observed findings to the general findings of VEXAS syndrome, AIP, and DAH is provided. This report underscores the rarity of specific pulmonary manifestations associated with VEXAS syndrome, contributing valuable insight to the limited literature available on this topic.

Aims: to test the measurement properties of the Portuguese version of the Commissioning for Quality in Rheumatoid Arthritis Patient-Reported Experience Measure (CQRA-PREM) for patients with rheumatoid arthritis (RA).

Methods: This cross-sectional clinical field study recruited adult patients with RA during rheumatology appointments of a Portuguese rheumatology center. Patients completed the Portuguese version of CQRA-PREM, composed of 7 domains and 24 questions. Sociodemographic characteristics, symptoms/disease duration, current treatment, Pain-Visual Analog Scale (VAS), Patient Global Assessment (PGA)-VAS and Health Assessment Questionnaire (HAQ) were also collected from the patient. Disease Activity Score for 28 joints with C-reactive Protein (DAS28-CRP) was recorded by the rheumatologist. The assessment of CQRA-PREM measurement properties followed the Consensus-based Standards for the Selection of Health Status Measurement Instruments (COSMIN) recommendations.

Results: A total of 61 patients with RA were included. The domains in which patients showed better experience were the "Needs and preferences", followed by "Coordination and Communication". The domain "Information, education and self-care" was an identified area of improvement for providing patient-centered care. Ceiling effects were found in four domains of the CQRA-PREM. Internal consistency of all domains was considered good (α>0.7). Homogeneity was considered good for each question in all domains analyzed (0.30≤rp≤0.70). The divergent validity of the PREM was good, revealing that the domains were not correlated (Pain-VAS, HAQ, DAS28-CRP) or only weakly (PGA-VAS) correlated with clinical outcomes.

Conclusions: The CQRA-PREM showed acceptable measurement properties and is a useful tool for evaluating quality of healthcare provided in daily practice, as perceived by RA patients in Portugal.

Introduction: Spondyloarthritis (SpA) is a group of chronic inflammatory diseases, often affecting women in reproductive age. These diseases can have a significant impact on the reproductive health of women. Preconception counseling and medication adjustments have shown to reduce flares and improve pregnancy outcomes in women with rheumatoid arthritis. However, in women with SpA data of the impact of preconception counselling on pregnancy outcomes is scarce. The aim of this study is to evaluate that.

Methods: In this retrospective multicentric study, data was collected from medical records of women who gave birth from 2020 to 2022. The study included 45 pregnancies, which were divided into two categories whether they received preconception consultation or not. Data was collected on patient characteristics, disease duration, medications used, and preconception counselling. Outcomes were divided into two groups: maternal and fetal outcomes.

Results: 30 out of 45 pregnancies (66.67%) had received preconception counselling, having a significantly lower percentage of flares occurring postpartum compared to the non-counselling group (36.6% vs 6.4%, p=0.031) and lower percentage of contraindicated medication during pregnancy (20.0 vs 0.0%, p=0.011).

Conclusion: Preconception counselling in women with SpA can increase the likelihood of medication adjustments before pregnancy and decrease the occurrence of flares postpartum. These findings suggest that preconception counselling should be implemented in the management of pregnant women with SpA to improve pregnancy outcomes. Further studies are needed to confirm the effectiveness of preconception counselling and to determine the optimal approach.

Pulmonary hypertension (PH) is one of the most feared complications of systemic sclerosis (SSc). There are currently specific drugs approved for PH group I (pulmonary arterial hypertension - PAH), but for PH related to lung disease (group III) the use of vasodilators is still controversial and not routinely recommended in patients with non-severe PH. However, SSc-PH-interstitial lung disease (ILD) has a poorer survival compared with SSc-PAH, making the management of these patients a challenge, ideally carried out in a reference centre. Herein we report the case of a a 45-year-old female with systemic sclerosis-myositis overlap syndrome, with documented lung involvement (ILD with fibrotic nonspecific interstitial/organizing pneumonia pattern), who was diagnosed with pre-capillary PH. She started sequential combination vasodilator therapy including parenteric prostanoid, with clinical benefit and without evidence of ILD worsening.