BMC Rheumatology最新文献

Background: Chronic recurrent multifocal osteomyelitis (CRMO) is a sporadic form of autoinflammatory bone disorders (ABDs) presenting with sterile chronic and/or recurrent and multiple sites of bone involvement. We aimed to describe our 10-year cohort of CRMO patients and analyze the characteristics and treatment approaches.

Methods: We retrospectively analyzed the data on patients with bone diseases at Children's Medical Center, Tehran University of Medical Sciences, Iran in the previous 10 years. The criteria for inclusion of patients as CNO/CRMO were mono-, oligo- or multifocal inflammatory bone lesions (osteomyelitis, osteitis, osteosclerosis) by imaging techniques; duration of complaints for > 6 weeks; and onset before 18 years of age.

Results: Thirty-nine patients were enrolled. Diagnosis of five patients were found compatible with monogenic ABDs. There were four sites of bone involvement per patient. The most common sites were vertebrae, pelvis, and tibia. Eight patients (23%) had dermatologic manifestations, including three psoriasis cases and one palmar pustulosis. All patients received NSAIDs, and 85% received it as first-line. Treatment was escalated to methotrexate or prednisolone when response was suboptimal. Out of 17 patients primarily treated only with NSAIDs, 47% remitted. In general, 84% of our patients are in remission: 36% without medication and 48% with medication.

Conclusion: Our CRMO patients showed an acceptable remission response to the current treatment regimen. Results of bone scintigraphy in suspected CRMO patients should be interpreted cautiously as an adjunct to clinical investigations. Special attention should be paid to extraosseous manifestations in suspected CRMO patients to avoid overlooking monogenic ABDs.

Clinical trial number: Not applicable.

Objectives: This study aimed to predict the knowledge of disease, quality of life, and related factors among rheumatoid arthritis patients.

Methodology: In this cross-sectional study, a total of 225 participants were recruited by convenience sampling from the RA outpatient clinics at Princess Basma Hospital and King Abdullah University Hospital in the north of Jordan between October 2023 and January 2024. The knowledge of RA was assessed using the adapted Rheumatoid Arthritis Knowledge Assessment Scale (RAKAS). Health-related quality of life (HRQoL) was evaluated using the generic EQ-5D-3 L instrument. Disease activity and remission were measured by DAS-28 ESR, which involved patient global assessment, ESR, and the number of swollen and tender joints. Data collection was achieved by face-to-face interviews and reviewing medical records. Predictors of disease knowledge and QoL were identified using quantile regression, One-way ANOVA, and multiple linear regression.

Results: The mean age of participants was 51.9 years, with 86.2% being female. Only 9.3% and 20.9% of patients, respectively, had "poor" or "low" knowledge, while 42.7% and 27.1% of patients, respectively, had " adequate " or "excellent" knowledge. Significant correlations of RA knowledge were observed with age, education level, duration of RA, and income. Specifically, younger patients, those with longer disease duration, higher education levels, and higher income demonstrated better knowledge of RA. Income and DAS score were significantly associated with the utility. Higher income levels were associated with an increase in utility. There was no association between disease knowledge and QoL in RA patients.

Conclusion: Adequate knowledge of the disease is prevalent among RA patients. Education level significantly affected both disease knowledge and quality of life. Interventions to enhance patient education and proper medication management are essential to improve health outcomes.

Clinical trial number: Not applicable.

Rheumatoid Arthritis (RA) is a chronic autoimmune disease that mostly breaks out at the joints. It further causes bone erosion and decreased life quality due to severe pain. Current drugs are mainly focused on reducing pain, but unable to terminate the disease progression. This study aims to determine the effect of diet types (Western, Vegan and Mediterranean) on RA progression. Some dietary supplements and drug administration (Huayu-Qiangshen-Tongbi formula or Leflunomide plus Methotrexate) in a six-month-period were also simulated to elucidate their effects on gut microbiota growth and exchange metabolite fluxes. The computational analyses showed that Haemophilus parainfluenzae had the highest growth rate in the RA community with the Western diet. Enterococcus faecalis was the most notable bacterial species considering butyrate exchange rates without any dependency on the diet; however diet type became important for Clostridium celatum for acetate and formate exchanges. Focal interactions for RA communities signify Mediterranean diet had the most homogeneous exchange flux distribution. With iron and ornithine supplementation, Clostridium celatum outshined the rest of the bacteria in the RA community with the potential being an RA biomarker. The Mediterranean diet could be studied further for drug administration studies since the bacterial species under this diet exhibited different outputs. In the near future, by utilizing the potential of the gut microbiota to be altered with diet, it might be possible to manipulate the progression of RA.

Objectives: Eosinophilic granulomatosis with polyangiitis (EGPA) involves systemic inflammation of small to medium vessels, with central nervous system (CNS) involvement being rare. While CT (computed tomography) and MRI (magnetic resonance imaging) are standard for diagnosing CNS involvement, digital subtraction angiography (DSA) is infrequently used. We present a unique EGPA case with CNS involvement and review EGPA CNS vascular variations.

Methods: We present a case of EGPA with CNS involvement, alongside a systematic review of the literature following PRISMA guidelines, querying three databases (PubMed/MEDLINE, SCOPUS, and Science Direct) up to September 2023 for case reports and series on EGPA with CNS involvement.

Results: A 43-year-old presented with wheezing, multifocal neuropathy, leukocytosis, eosinophilia, positive ANA, and elevated CRP. Imaging revealed lung abnormalities. CT and MRI showed cerebral infarcts. CTA was negative, whereas DSA revealed bilateral segmental narrowing of anterior cerebral artery (ACA) branches and middle cerebral artery (MCA) branches. EGPA was confirmed, and treatment with steroids, cyclophosphamide, and azathioprine, led to remission. A systematic literature review of 27 EGPA cases with CNS involvement found a mean age 54.22 years, with common symptoms including extremity weakness (n = 8) and paresthesia (n = 5). Imaging techniques included MRI (n = 21), CT (n = 11), angiogram (n = 8), MRA (n = 4), CTA (n = 4), and MRV (n = 2), revealing stenosis of the bilateral ACA, vertebral artery, MCA, and basilar artery.

Conclusion: Our findings suggest a potentially novel role for angiographic imaging in the comprehensive assessment of cerebrovascular involvement in EGPA.

Objective: To investigate the relationship between Life's Essential 8 (LE8) and its individual constituents and the risk of rheumatoid arthritis (RA).

Methods: This cross-sectional study included participants aged 20 years and older from the NHANES database from 2005 to 2018. LE8 scores and scores for each of the LE8 components including diet, physical activity, nicotine exposure, sleep health, body mass index, lipids, blood glucose and blood pressure, were classified as cardiovascular health (CVH) scores of low (0-49), moderate (50-74), and high (75-100). Multivariate logistic regression was used to assess the association between LE8 scores and individual LE8 metric scores and the risk of RA. A curve-fitting model was used to assess the dose-response relationship between LE8 scores and RA risk.

Results: Of the 17,943 subjects (mean age: 46.10 ± 16.99 years; 48.33% males) included, 1233 were identified as having RA. After multivariate adjustment, participants with an intermediate or high LE8 score were associated with a reduced risk of RA compared to those with a low LE8 score (intermediate LE8 score: OR = 0.66, 95% CI = 0.62-0.71; high LE8 score: OR = 0.66, 95% CI = 0.62-0.71). Similar risk patterns were found for diet, nicotine exposure, sleep health, body mass index, and blood glucose. The dose-response relationship between LE8 score and RA risk was nonlinear.

Conclusions: Higher scores of CVH, assessed by Life's Essential 8, were significantly associated with a lower risk of RA. Maintaining an ideal CVH score in the general population may be beneficial in preventing RA.

Background: Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by increased vascular permeability leading to third-spacing of fluids and protein. Drug-induced hypersensitivity reactions can mimic SCLS clinically and radiologically.

Case presentation: A 42-year-old Vietnamese man developed abdominal distension, facial edema, and dyspnea after initiation of Helicobacter pylori eradication therapy. Imaging revealed pleural, pericardial, and peritoneal effusions, periportal edema, and interstitial pulmonary edema. Laboratory results showed hyponatremia, hypoalbuminemia, and mild anemia. Autoimmune screening revealed ANA positivity (1:80, speckled) and lupus anticoagulant, though extractable nuclear antigens were negative. The patient improved rapidly with corticosteroids and antihistamines.

Conclusion: This case suggests a probable drug-induced systemic hypersensitivity reaction mimicking capillary leak syndrome, occurring in a patient with latent immune dysregulation. Awareness of this presentation may facilitate early recognition and appropriate immunomodulatory treatment while avoiding unnecessary interventions.

Background: Idiopathic inflammatory myopathies (IIM) are a diverse group of autoimmune diseases characterized primarily by muscle weakness and systemic involvement, which can include interstitial lung disease (ILD). ILD is a serious complication in IIM, significantly affecting patient prognosis and quality of life. Early identification of IIM patients at risk for developing ILD is crucial for timely intervention and personalized treatment, yet the factors contributing to this risk remain inadequately defined.

Methods: This retrospective study analyzed medical records of 130 patients with IIM from the First Affiliated Hospital of Xinxiang Medical University, China, between August 2018 and July 2023. Patients were categorized into two groups: IIM with interstitial lung disease (IIM-ILD, n = 75) and IIM without ILD (n = 55). We collected and analyzed demographic, clinical, and laboratory data, including specific autoantibody tests. Multivariate logistic regression identified independent predictors of ILD, and a nomogram was developed to evaluate ILD risk based on significant factors.

Results: This retrospective study analyzed 130 patients with IIM, including 75 with interstitial lung disease and 55 without ILD. The IIM-ILD group was significantly older (58.4 vs. 48.3, p = 0.052) and had higher frequencies of respiratory symptoms including dyspnea (61.3% vs. 14.9%, p < 0.001) and cough (54.7% vs. 10.9%, p < 0.001). Key laboratory differences included elevated ESR (26.5 vs. 10.0 mm/H, p < 0.001), CRP (3.44 vs. 1.64 mmol/L, p = 0.013), and IgG (12.5 vs. 10.9 g/L, p = 0.006), along with lower ALT (29.0 vs. 44.0 U/L, p = 0.001) and AST (32.0 vs. 45.0 U/L, p = 0.021) in the IIM-ILD group. Anti-Jo-1 antibodies were more prevalent in IIM-ILD patients (18.7% vs. 5.5%, p = 0.027). Multivariate analysis identified ESR (OR = 1.063, 95% CI:1.012-1.117, p = 0.015), AST (OR = 0.985, 95% CI:0.970-1.000, p = 0.047), and IgG (OR = 1.191, 95% CI:1.025-1.383, p = 0.022) as independent predictors. These factors, combined with dyspnea and anti-Jo-1 status, were incorporated into a predictive nomogram model. The nomogram demonstrated excellent discrimination (AUC = 0.891, 95% CI:0.836-0.947) with sensitivity of 79.7% and specificity of 82.6%. Calibration curves showed good agreement between predicted and observed outcomes (Hosmer-Lemeshow test, p = 0.779). Decision curve analysis confirmed the model's clinical utility across a wide range of threshold probabilities. This comprehensive model provides clinicians with a practical tool for early identification of IIM patients at high risk for ILD development.

Conclusion: Elevated ESR and CRP levels, in conjunction with lower AST levels, alongside the presence of anti-Jo-1 antibodies and the manifestation of dyspnea are significant biomarkers associated with the risk of developing IIM-ILD. This predictive model enhances early diagnostic cap

Background: Rheumatoid arthritis is an autoimmune disease that can cause joint destruction, pain, loss of function, and reduced quality of life. Recent advancements in treatment have made it possible to control the impacts of this once-debilitating disease through early intervention. While numerous studies have examined barriers to rheumatoid arthritis care, no review has synthesized sociodemographic and economic factors across high-, upper middle-, and lower middle-income countries. This gap in the literature highlights the need for a comprehensive review that informs global health interventions. This review explores sociodemographic and economic barriers to initial specialist care for patients with rheumatoid arthritis.

Methods: The review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) guidelines. A search of CINAHL, MEDLINE, Scopus and Emcare was completed in May 2024.

Results: Of the 5165 studies identified through the literature search, 121 full-text articles were reviewed, and 25 studies examining sociodemographic and economic barriers to specialist care were selected for analysis. A total of 17 high-income, one upper middle-income and seven lower middle-income countries were represented. Low socioeconomic status, low income and rurality were consistently reported as barriers to initial rheumatologist appointments across all countries in this review.

Conclusion: These findings underscore the importance of addressing common barriers such as low socioeconomic status and rurality in global health interventions. Future large prospective studies are essential to better understand the relationship between sociodemographic factors and timely access to care.

Background: To investigate a patient-level single imputation approach for patient reported outcomes (PROs) that express similar contents or associated PROs, where a PRO whose value is missing at a particular timepoint is substituted by another PRO whose value is available at the same timepoint.

Methods: We performed a simulation study on registry-based spondyloarthritis data to explore the potential interchangeability between the patient pain (PPA) and fatigue (PFA) assessment scores and relevant Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) individual questions, and between PPA, PFA and patient global assessment (PGA). Performance was assessed per imputation method in terms of relative bias and coverage. Sample size, level of missingness and missing data pattern were included as parameters in the simulations.

Results: All applied scenarios to interchange PPA with BASDAI question 2 (axial pain), BASDAI question 3 (peripheral joint pain/swelling) or their average failed. Interchangeability between PFA and BASDAI question 1 (fatigue/tiredness) was acceptable for partially (up to 50%) missing data. When interchanging patient assessment scores (PPA, PFA and PGA), we observed inconsistent results in terms of performance. The performance of the applied methods depended on the sample size and the level of missingness, but not heavily on the underlying missing data pattern.

Conclusions: Interchanging PFA and the BASDAI fatigue question was justified for partially missing data, while interchangeability between PPA, PFA and PGA, and between PPA and the BASDAI pain questions was not advised. Our findings suggest that registering patient assessment scores and BASDAI questions is recommended.

Background: Rheumatoid arthritis (RA) is a chronic autoimmune disorder characterized by joint destruction and systemic inflammation, both of which significantly impair patients' quality of life. Mild cognitive impairment (MCI), a reversible precursor to dementia, is increasingly prevalent among elderly RA patients. Early identification of MCI in this population allows for timely interventions to slow cognitive decline.

Objective: This study aims to identify independent risk factors for MCI in elderly patients with RA and to develop a predictive nomogram.

Methods: We enrolled 378 elderly RA patients, aged 60 to 80 years, from Xi'an Fifth Hospital between December 2023 and December 2024. Cognitive function was assessed using the Montreal Cognitive Assessment (MoCA), with scores ranging from 20 to 26 indicating MCI. We analyzed demographic, clinical, and laboratory data to identify risk factors through logistic regression and constructed a nomogram. The model's performance was evaluated using receiver operating characteristic (ROC) curves, calibration plots, and decision curve analysis (DCA).

Results: Among the 378 patients, 94 (24.87%) were classified in the RA-MCI group. Multivariate analysis identified the course of disease (COD) (OR = 1.07, 95% CI: 1.03-1.10), elevated Disease Activity Score-28 (DAS28) (OR = 1.31, 95% CI: 1.13-1.53), high C-reactive protein (CRP) levels (OR = 1.01, 95% CI: 1.01-1.02), and osteoporosis (OP) (OR = 1.88, 95% CI: 1.14-3.13) as independent risk factors. The nomogram demonstrated moderate discrimination (AUC = 0.750, 95% CI: 0.696-0.805) and clinical utility.

Conclusion: The COD, OP, DAS28, and CRP levels are key predictors of MCI in elderly RA patients. The proposed nomogram provides a practical tool for early risk stratification, facilitating targeted interventions to delay cognitive decline.

Trial registration: This study conformed to the principles outlined in the Declaration of Helsinki and received approval from the Medical Ethics Committee of Xi'an Fifth Hospital (Approval No.: [2023] Ethics Review 55). Additionally, the trial was registered with the Chinese Clinical Trial Registry (Registration No.: ChiCTR2300077337, Registration Date: 2023-11-01). Written informed consent was obtained from all individual participants included in the study.