BMC Rheumatology最新文献

Background: Medical treatment for children with Juvenile Idiopathic Arthritis (JIA) has improved radically since the development of biological disease-modifying antirheumatic drugs. However, children suffer from pain and anxiety, and parents often experience loneliness and lack of support. Some parents reported that information provided at the time their child was diagnosed could be difficult to assimilate. Therefore, the aim of this study was to develop a Juvenile Arthritis Support Program (JASP-1) for children recently diagnosed with JIA and their parents. Moreover, the aim was to explore patients´ and parents´ experiences with JASP-1 and its potential impact on patients´ physical health.

Methods: JASP-1 included seven patient- and family-centered clinical visit from time of diagnose and one year ahead. Data were collected from a study-specific questionnaire answered by children and their parents after participation in JASP-1 and from the pediatric rheumatology register. The study-specific questionnaire explored participants´ experience with the care they received during their first year with JIA. Registry and questionnaire data from the intervention (JASP-1) group was compared to a control group.

Results: The analysis revealed that children and parents who completed JASP-1 were more satisfied with the care they had received during their first year with JIA than the control group. The results also showed that children who completed JASP-1 were assessed as having better overall health after 12 months, than children in the control group (JASP-1 = mean 4.33, 95% Confidence Interval (CI) 4.17 - 4.46), (Control = mean 3.68, 95% CI 3.29 - 4.06), (p = 0.002). Moreover, children in the JASP-1 group had less disease impact on daily life (JASP-1 = mean 0.15, 95% CI 0.07 - 0.24) (Control = mean 0.40, 95% CI 0.13 - 0.67), (p = 0.017) and less active joints than the control group (JASP-1 = mean 0.62, 95% CI 0.35 - 1.58), (Control = mean 0.87, 95% CI 0.18 - 1.56), (p = 0.054).

Conclusion: A support program like JASP-1 could be an effective way of not only supporting children newly diagnosed with JIA and their parents psychologically but may also increase children's overall physical health and improve quality of care within pediatric rheumatology.

Trial registration: Retrospectively registered in ClinicalTrials.gov, the 13th of February with ID NCT06284616.

Background: to detect the role of procalcitonin, erythrocyte sedimentation rate to c-reactive protein (ESR/CRP) ratio, neutrophils-to-lymphocyte ratio (NLR), and platelets-to-lymphocyte ratio (PLR) in the diagnosis of infection in systemic lupus erythematosus (SLE) patients with fever, their diagnostic value to differentiate between infection and disease activity, and their correlation with disease activity.

Methods: Forty SLE patients and forty healthy control cases were included in the study. Disease activity was assessed by the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K), and quality of life was assessed by Lupus QoL. A bacterial infection was detected by clinical symptoms and positive culture results. Laboratory tests were done for all patients and controls: complete blood count (CBC), ESR, CRP, and procalcitonin (PCT). NLR, PLR, and ESR/CRP ratios were calculated.

Results: There was a statistically significant difference between infected SLE patients and non-infected SLE patients regarding PCT (p < 0.001), ESR (p = 0.002), CRP (p = 0.005), ESR/CRP ratio (0.002), and NLR (p = 0.023). PCT, ESR, CRP, and NLR were positively correlated with the presence of infection in SLE patients, while the ESR/CRP ratio was negatively correlated. There was no significant correlation with the SLEDAI-2 K score. Logistic regression analysis revealed that PCT was the best significant predictor of infection (OR 224.37, 95% CI 8.94-5631.35). PCT was a good predictor of infection, with a cut-off value of 0.90 ng/ml, which gave the best combination of sensitivity (84.62%) and specificity (85.71%).

Conclusion: PCT, ESR/CRP ratio, and NLR provide good diagnostic markers for the diagnosis of infection and can distinguish between infection and disease flare in SLE patients with fever.

Background: Interstitial lung disease (ILD) is an increasingly recognized complication of rheumatoid arthritis (RA) and is associated with significant morbidity and mortality. Many risk factors for RA-related ILD were reported. The current study aims to explore the features and risk factors of Saudi patients with RA-ILD.

Methods: This is a multicenter, retrospective, observational study of patients with RA-ILD. Clinical and radiological data from patients with RA-ILD were obtained from electronic medical records, including demographics, clinical characteristics, laboratory tests, pulmonary function tests, ECHO, and HRCT images.

Result: Out of 732 patients, 57 had RA-ILD. The mean age at the time of ILD diagnosis was 61.9 (± 12.2) years. RA-ILD diagnosis was significantly less among females (p = 0.008). Patients who ever smoked had significantly more RA-ILD (p < 0.001). Patients with RA-ILD were more likely to present with medical comorbidities, namely diabetes (p < 0.001), hypertension (p < 0.001), ischemic heart disease (p < 0.001), and osteoarthritis (p = 0.030). The multivariate analysis revealed that the age (OR: 1.035, 95% CI: 48.45-52.86, p = 0.0001); gender (OR: 2.581, CI: 1.77-1.86, p = 0.001), DM (OR: 2.498, 95% Cl: 1.65-1.76, P = 0.0001), HTN (OR: 1.975, 95% Cl: 1.61-1.74, P = 0.019), IHD (OR: 6.043, 95% Cl: 1.89-1.93, P = 0.0001) have a significant positive association with RA-ILD. No significant differences were observed between seropositive parameters with or without RA-ILD (p > 0.05). The most common symptoms of RA-ILD were cough (55.6%) and dyspnea (30.2%), and the most common ILD pattern was Non-specific Interstitial Pneumonia (NSIP) (55.6%) followed by Usual Interstitial Pneumonia (UIP) (38.9%). Traction bronchiectasis (75.5%) and glass ground opacities (73.6%) were also observed. The mean FVC and DLCO at baseline were 64.6% and 53.3%, respectively.

Conclusion: In this cohort of patients, Saudi RA-ILD patients had a predominant NSIP pattern conversely to what is seen globally. These findings could be explained by the lower rates of smoking in our patient population. Future prospective national studies are needed to confirm the current findings and better evaluate RA-ILD epidemiology and risk factors.

Background: Systemic lupus erythematosus (SLE) may result in great impact on patients' quality of life, social relationships, and work productivity. The use of patient-reported outcome measures (PROMs) in routine care could help capture disease burden to guide SLE management and optimize disease control. We aimed to explore the current situation, appropriateness, and feasibility of PROMs to monitor patients with SLE in routine care, from healthcare professionals' and patients' perspectives.

Methods: A scientific committee developed a Delphi questionnaire, based on a focus group with patients and a literature review, including 22 statements concerning: 1) Use of PROMs in routine care (n = 2); 2) PROMs in SLE management (n = 13); 3) Multidisciplinary management of patients with SLE (n = 4), and 4) Aspects on patient empowerment (n = 3). Statements included in Sects. 2-4 were assessed from three perspectives: current use, appropriateness, and feasibility (with currently available resources). For each statement, panellists specified their level of agreement using a 7-point Likert scale. A consensus was reached when ≥ 70% of the panellists agreed (6,7) or disagreed (1,2) on each statement.

Results: Fifty-nine healthcare professionals and 16 patients with SLE participated in the Delphi-rounds. A consensus was reached on the value of PROMs to improve SLE management (83%) and the key role of healthcare professionals (77%) and the need for a digital tool connected to the electronic medical record (85%) to promote and facilitate PROMs collection. PROMs most frequently used in clinical practice are pain (56%), patient's global assessment (44%) and fatigue (39%), all on visual analogue scales. Panellists agreed on the need to implement multidisciplinary consultation (79%), unify complementary tests (88%), incorporate pharmacists into the healthcare team (70%), and develop home medication dispensing and informed telepharmacy programmes (72%) to improve quality of care in patients with SLE. According to panellists, patient associations (82%) and nurses (80%) are critical to educate and train patients on PROMs to enhance patient empowerment.

Conclusions: Although pain, fatigue, and global assessment were identified as the most feasible, PROMs are not widely used in routine care in Spain. The present Delphi consensus can provide a road map for their implementation being key for SLE management.

Background: Rheumatoid arthritis (RA) is a chronic, systemic, inflammatory joint disease, that influences patients' health in different ways, including physical, social, emotional, and psychological aspects. The goal of rheumatology care is to achieve optimal health and personalised care and therefore, it is essential to understand what health means for patients in the early course of RA. The aim of this study was to describe the understanding of health among patients with early RA.

Methods: The study had a descriptive qualitative design with a phenomenographic approach. Phenomenography is used to analyse, describe, and understand various ways people understand or experience a phenomenon, in this study, patients' understandings of health. Individual semi-structured interviews were conducted with 31 patients (22 women and nine men, aged (38-80) with early RA, defined as a disease duration of < 1 year, and disease-modifying anti-rheumatic drugs (DMARDs) for 3-7 months. The phenomenographic analysis was conducted in 7 steps, and the outcome space presents the variation in understanding and the interrelation among categories. In accordance with the European Alliance of Associations for Rheumatology's (EULAR) recommendations, a patient research partner participated in all phases of the study.

Results: The analysis revealed four main descriptive categories: 'Health as belonging' was described as experiencing a sense of coherence. 'Health as happiness' was understood as feeling joy in everyday life. 'Health as freedom' was understood as feeling independent. 'Health as empowerment' was understood as feeling capable. Essential health aspects in early RA are comprised of a sense of coherence, joy, independence, and the capability to manage everyday life.

Conclusions: This study revealed that patients' perception of health in early RA encompasses various facets, including a sense of belonging, happiness, freedom, and empowerment. It highlighted that health is multifaceted and personal, emphasizing the importance of acknowledging this diversity in providing person-centred care. The findings can guide healthcare professionals to deepen patients' participation in treatment goals, which may lead to better treatment adherence and health outcomes.

Introduction: Systemic sclerosis (SSc) is a rare, complex autoimmune rheumatic disease with multiple factors that contribute to pain. People with SSc emphasize the effect pain has on their quality of life, but no studies have systematically examined the frequency and relative importance of different SSc pain sources, patterns of pain from different sources, and pain management experiences. Our objectives are to (1) develop a tool, jointly with researchers, health care providers, and patients, to map sources of pain in SSc, determine patterns of pain from different sources, and understand pain management experiences; and (2) administer the final tool version to participants in the large multinational Scleroderma Patient-centered Intervention Network (SPIN) Cohort.

Methods: First, we will use validated pain assessment tools as templates to develop an initial version of our pain assessment tool, and we will obtain input from patient advisors to adapt it for SSc. The tool will include questions on pain sources, pain patterns, pain intensity, pain management techniques, and barriers to pain management in SSc. Second, we will conduct nominal group technique sessions with people living with SSc and health care providers who care for people with SSc to further refine the tool. Third, we will conduct individual usability testing sessions with SPIN Cohort participants. Once the tool has been finalized, we will administer it to individuals in the multinational SPIN Cohort, which currently includes over 1,300 active participants from 54 sites in 7 countries. We will perform unsupervised clustering using the KAy-Means for MIxed LArge data (KAMILA) method to identify participant subgroups with similar profiles of pain sources (present or absent) and to evaluate predictors of subgroup membership. We will use latent profile analysis to identify subgroups of participants with similar profiles based on pain intensity scores for each pain source and evaluate predictors.

Discussion: Once completed, our pain assessment tool will allow our team and other researchers to map sources of pain in SSc and to understand pain management experiences of people living with SSc. This knowledge will provide avenues for studies on the pathophysiology of pain in SSc and studies of interventions to improve pain management.

Background: In 2020, Nintedanib (NTB), a tyrosine kinase inhibitor, was the first drug approved worldwide for treating progressive fibrosing interstitial lung disease (PF-ILD). This study evaluated the efficacy and safety of NTB in Japanese patients with CTD-associated PF-ILD in a real-world setting, as there are few reports on this topic. We also evaluated the efficacy and safety of combination therapy with NTB and immunosuppressive agents (IS).

Methods: CTD-associated PF-ILD patients receiving NTB at our institution were included in this retrospective study. To evaluate the efficacy and safety of NTB, we investigated changes in forced vital capacity (FVC) (%), diffusing capacity for carbon monoxide (DLCO) (%), monthly change in FVC (%/month), serum Krebs von den Lungen-6 (KL-6) levels (U/mL) before and after NTB treatment, and adverse events (AEs) during NTB treatment. Moreover, to evaluate the efficacy of the NTB + IS combination therapy, we divided the patients into two groups: one received only NTB (NTB group), and the other received both NTB and IS (NTB + IS group) following the diagnosis of CTD-associated PF-ILD. We analyzed the differences in the changes of these variables between the two groups.

Results: Twenty-six patients with CTD-associated PF-ILD were included. After NTB treatment, there were no significant deteriorations in FVC (%) and DLCO (%), while the monthly change in FVC (%/month) significantly increased (p < 0.001). The changes in FVC (%) and the monthly change in FVC (%/month) were significantly greater in the NTB + IS group than in the NTB group. Following NTB treatment, the mean serum KL-6 levels significantly decreased (p < 0.001). AEs associated with NTB in this study were similar to those in previous clinical trials, and there was no significant difference in the incidence of AEs between the two groups.

Conclusions: This study demonstrates that NTB is an effective medication for slowing the progression of CTD-associated PF-ILD in real-world settings. NTB + IS combination therapy for CTD-associated PF-ILD may be more effective than NTB alone in slowing the progression of CTD-associated PF-ILD.

Introduction: Recent works in the scientific literature reported the role of C reactive protein to albumin ratio (CAR), neutrophil to lymphocyte ratio (NLR) and platelet to lymphocyte ratio (PLR) as biomarkers of disease activity in rheumatic diseases.

Objectives: To investigate the role of CAR, PLR and NLR as potential markers of disease activity in children with non-systemic JIA (nsJIA) and their correlation with the risk of persistent disease activity of flare during follow up.

Methods: Our prospective, cross-sectional study involved 130 nsJIA patients (74 with active disease and 56 with inactive disease according to Wallace criteria) and 62 healthy controls. Demographic, clinical and laboratory data were collected at baseline (T0) and at 3 (T1), 6 (T2), 12 (T3) and 18 months (T4) during follow up. Disease activity was evaluated through Juvenile Arthritis Disease Activity Score (JADAS-27).

Results: At baseline, CRP and CAR were higher in patients than in controls (p = 0.046), while no differences were found for NLR and PLR. However, there was no positive correlation between CAR, NLR, PLR and JADAS-27 in JIA patients. To better investigate the role of CAR, NLR and PLR as markers of disease activity, we used a generalized estimating equation (GEE) model, applied to all patients either with or without active disease. According to this analysis, CAR and NLR baseline levels were predictive of higher risk of disease activity at 6 months follow up (p < 0.001).

Conclusions: CAR and NLR could indicate persistent disease activity in patients with JIA. Their predictive value could be increased by their combined use and by the observation of their trend during follow up, since increasing CAR values over time could predict a disease flare in the brief time.