Journal of Rheumatic Diseases最新文献

Objective: The aim of this study was to evaluate the prevelance of lumbosacral transitional vertebrae (LSTV) in patients with axial spondyloarthritis (ax-SpA), and to explore the relationship of the presence of LSTV with the burden of disease.

Methods: A total of 177 patients classified with ax-SpA according to ASAS (Assessment of Spondyloarthritis International Society) criteria who were admitted to Selçuk University Medical Faculty rheumatology outpatient clinic were included, consecutively. Demographic, clinical, and laboratory data were recorded. LSTV was evaluated on AnteroPosterior (AP) pelvic radiographs and AP lumbar radiographs according to the Castellvi classification.

Results: Of 177 patients with axSpA, 99 (55.9%) were radiographic axSpA (r-axSpA). LSTV was detected in 51 (28.8%) patients with ax-SpA (with the frequency of 32.3% in r-axSpA and 24.4% in non-r-axSpA [nr-axSpA] [p>0.05]). Most frequent LSTV subtype was type 1 (32 of 51 [62.7%]). Thirty-two (62.7%) of 51 patients with LSTV were r-axSpA, and 19 (37.3%) patients with LSTV were nr-axSpA (p=0.245). The demographic characteristics of the group with and without LSTV were similar. There was no significant difference in terms of VAS, BASDAI, BASFI, ASDAS-CRP, BASMI, HAQ, EQ-5D-3L scores and no difference in disease activity in terms of presence of LSTV in patients with ax-SpA.

Conclusion: The prevalence of LSTV in patients with ax-SpA was 28.8%, consistent with some population based studies in the literature. There was no difference between patients with and without LSTV in terms of burden of disease in patients with ax-SpA.

Chimeric Antigen Receptor (CAR) T-cell therapy, revolutionary in treating hematological malignancies, is emerging as a promising approach for systemic autoimmune rheumatic diseases (SARDs). This review examines the potential of CAR T-cell therapy in treating conditions such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and idiopathic inflammatory myopathies (IIMs). The evolution of CAR T cells technology, from first to fifth generation, has enhanced its efficacy and persistence. Early clinical studies in SARDs have shown encouraging results, with some patients achieving drug-free remission. CD19-targeted CAR T cells have demonstrated significant B-cell depletion and clinical improvement in patients with SLE, SSc, and IIMs. Despite promising outcomes, challenges remain, including cytokine release syndrome and the need for careful patient selection. Future directions include exploring dual-targeting CARs, chimeric autoantibody receptors (CAARs), and alternative cell sources like γδ T cells, regulatory T cells, natural killer cells. The integration of CAR-based cell therapy into treatment paradigms of patients with SARDs requires further research to optimize efficacy, mitigate side effects, and identify suitable target biomarkers. While hurdles exist CAR-based cell therapy holds the potential to revolutionize management of patients with SARDs, offering hope for long-term, drug-free remission in these complex autoimmune conditions.

Objective: Previous studies reported that abatacept (ABT) decreased autoantibodies in early rheumatoid arthritis (RA) patients. We investigated the impact of ABT, and other biological disease-modifying anti-rheumatic drugs (bDMARDs) on autoantibody levels in established RA patients.

Methods: This prospective observational study included 50 RA patients treated with ABT and 115 RA patients treated with non-ABT bDMARDs. Serum levels of anticitrullinated peptide antibodies (ACPA), immunoglobulin (Ig) M-rheumatoid factor (IgM-RF), IgG-RF, and anti-agalactosyl IgG antibody (anti-Gal (0) IgG) were measured at baseline and after 48 weeks of treatment.

Results: After propensity score matching, 25 patients with ABT and 25 patients with non-ABT were finally analyzed. Disease activity score in 28 joints using C-reactive protein significantly decreased in both ABT group (4.5 to 3.3, p<0.01) and non-ABT group (4.4 to 2.5, p<0.01) after 48 weeks treatment. In ABT group, median titers at baseline and 48 weeks were 62.7 and 57.8 U/mL for ACPA (p=0.22), 35.0 and 39.0 IU/mL for IgM-RF (p=0.21), 0.5 and 0.5 IU/mL for IgG-RF (p=0.19), and 50.4 and 53.5 AU/mL for anti-Gal (0) IgG (p=0.22), respectively. Changes of all autoantibody titer were not significant in ABT group. Non-ABT group showed significant decreases in ACPA (baseline 143.0 to 57.8 U/mL at week 48, p=0.03), IgM-RF (50.0 to 37.0 IU/mL, p<0.01), and anti-Gal (0) IgG (93.2 to 61.8 AU/mL, p<0.01) except IgG-RF (0.6 to 0.5 IU/mL, p=0.22).

Conclusion: Autoantibody-lowering effect of ABT was not strong in established RA patients in our study.

Rheumatoid arthritis (RA) is a chronic autoimmune disease characterized by synovial inflammation and joint destruction. Despite advances in biologic therapies targeting inflammatory mediators such as tumor necrosis factor (TNF)-α, interleukin (IL)-6, Janus kinase (JAK), and B cells, many patients do not respond adequately, emphasizing the need for deeper insights into RA pathogenesis. Research highlights the intricate interplay of genetic and epigenetic factors driving immune dysregulation. The breakdown of immune tolerance, often initiated in mucosal sites such as the gut, lung, and oral cavity, promotes the citrullination of antigens, leading to anti-citrullinated protein antibody production and subsequent immune activation. Single-cell and multi-omics approaches have shed light on underexplored immune cell types, such as T peripheral helper cells, CD4+/CD8+ cytotoxic T cells, and autoreactive B cells, broadening the understanding beyond traditionally studied Th17, Th1 cells, macrophages, and fibroblast-like synoviocytes. Future basic research in RA should prioritize elucidating the mechanisms behind peripheral tolerance breakdown, the pathogenesis of seronegative RA, and the molecular pathways driving refractory and recurrent disease. Moreover, leveraging multi-omics approaches to dissect disease heterogeneity will be pivotal for advancing personalized treatment strategies and improving long-term outcomes in RA patients.

Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is a rare disorder characterized by features of both relapsing polychondritis (RP) and Behçet's disease (BD), with multi-organ involvement, including inflammation and destruction of cartilaginous tissues. This report describes a patient with MAGIC syndrome who responded to immunosuppressive therapy for long-lasting laryngeal inflammation and provides the first description of a patient with MAGIC syndrome positive for human leukocyte antigen (HLA)-A26. Here we present a 49-year-old male with recurrent oral and genital ulcers, hoarseness, and swallowing difficulties. Laryngoscopy showed bilateral vocal fold immobility, and contrast-enhanced magnetic resonance imaging (MRI) demonstrated inflammation in the cricoid, arytenoid, and auricular cartilages. The patient was diagnosed with MAGIC syndrome based on the presence of both RP and BD criteria. Treatment with high-dose corticosteroids and adalimumab biosimilar resulted in significant improvement in hoarseness and MRI findings of cartilage inflammation. This case highlights the effective use of immunosuppressive therapy for managing long-lasting laryngeal involvement in MAGIC syndrome, potentially avoiding the need for invasive interventions such as tracheostomy.

Systemic sclerosis (SSc), or scleroderma, is a complex autoimmune connective tissue disease characterized by autoimmunity, vasculopathy, and progressive organ fibrosis, leading to severe organ dysfunction. The disease begins with a vascular injury triggered by autoimmune responses and environmental factors against a backdrop of genetic predisposition. This injury impairs angiogenesis and vasculogenesis, resulting in capillary loss and arteriolar constriction, which promotes immune cell infiltration and sustained inflammation within affected tissues. These vascular anomalies cause severe complications, including pulmonary artery hypertension, scleroderma renal crisis, and skin ulcers. Chronic inflammation fosters persistent fibroblast activation, resulting in extensive fibrosis that defines SSc. This review synthesizes the latest research on pathogenesis of SSc, highlighting the shift from fundamental research to a precision therapeutic approach. It explores the potential of technologies like flow cytometry and single-cell RNA sequencing to investigate pathogenic cell subtypes. These platforms integrate transcriptomic, genomic, proteomic, and epigenomic data to uncover insights into the underlying mechanisms of SSc pathogenesis. This review advocates for a multidisciplinary, patient-centric approach that harnesses recent scientific advances, directing future SSc research toward personalized and precise interventions.

Objective: CT-P13, a biosimilar of infliximab, is widely used for treating ankylosing spondylitis (AS). However, the formation of anti-drug antibodies (ADAs) can reduce its efficacy. This study aimed to identify risk factors associated with high ADA levels in AS patients treated with CT-P13.

Methods: A prospective observational study enrolled patients with intravenous CT-P13. Clinical data and disease activity was assessed at baseline, 24 weeks, and 54 weeks after CT-P13 treatment. Blood concentrations of CT-P13 and ADAs were measured at 24 and 54 weeks, and their correlation was investigated. Patients were grouped by ADA levels at 54 weeks. Univariable and multivariable logistic regression identified factors associated with high ADA concentrations.

Results: A total of 34 patients was enrolled. Significant decreases in Bath Ankylosing Spondylitis Disease Activity Index and Bath Ankylosing Spondylitis Functional Index scores were observed relative to baseline after 24 weeks of CT-P13 therapy. Serum concentrations of CT-P13 and ADA levels increased following treatment. The median serum CT-P13 concentration was 17.6 [12.8, 22.7] µg/mL at 24 weeks and 23.5 [11.7, 34.2] µg/mL at 54 weeks. ADA levels were 6.7 [6.5, 9.1] AU/mL at 24 weeks and 11.4 [9.0, 28.4] AU/mL at 54 weeks. The serum concentrations of CT-P13 and ADA exhibited a negative correlation. In multivariable analysis, current smoking was associated with high ADA production at 54 weeks.

Conclusion: Smoking is identified as a significant risk factor for elevated ADAs in AS patients treated with CT-P13. The findings underscore the importance of smoking-cessation strategies in the management of AS patients.

Rheumatic diseases, such as rheumatoid arthritis (RA), osteoarthritis (OA), and spondyloarthritis (SpA), present diagnostic and management challenges due to their impact on connective tissues and the musculoskeletal system. Traditional imaging techniques, including plain radiography, ultrasounds, computed tomography, and magnetic resonance imaging (MRI), play a critical role in diagnosing and monitoring these conditions, but face limitations like inter-observer variability and time-consuming assessments. Recently, deep learning (DL), a subset of artificial intelligence, has emerged as a promising tool for enhancing medical imaging analysis. Convolutional neural networks, a DL model type, have shown great potential in medical image classification, segmentation, and anomaly detection, often surpassing human performance in tasks like tumor identification and disease severity grading. In rheumatology, DL models have been applied to plain radiography, ultrasounds, and MRI for assessing joint damage, synovial inflammation, and disease progression in RA, OA, and SpA patients. Despite the promise of DL, challenges such as data bias, limited explainability, and the need for large annotated datasets remain significant barriers to its widespread adoption. Furthermore, human oversight and value judgment are essential for ensuring the ethical use and effective implementation of DL in clinical settings. This review provides a comprehensive overview of DL's applications in rheumatologic imaging and explores its future potential in enhancing diagnosis, treatment decisions, and personalized medicine.

Objective: Using a nationally representative cohort of medical claims data in Korea, this study aimed to analyze the association between the use of various anti-rheumatic agents and the risk of acute myocardial infarction (AMI) in patients with rheumatoid arthritis (RA).

Methods: This nested case-control study used the Korean Health Insurance Review and Assessment data of 35,133 patients newly diagnosed with RA between 2011 and 2020. Incident AMI patients were identified and matched at a 14 ratio with randomly selected controls. The usage of anti-rheumatic agents was measured from the date of RA diagnosis to the index date and stratified based on exposure time and duration. The risk of AMI associated with each anti-rheumatic agent was estimated using conditional logistic regression, adjusted for comorbidities and concomitant drug use.

Results: Of the 35,133 patients with RA, 484 were diagnosed with AMI. In total, 484 AMI patients and 1,924 controls with newly diagnosed RA were included in the analysis. Current exposure and long-term exposure to glucocorticoids (adjusted odds ratio [aOR] 2.301, 95% confidence interval [CI] 1.741~3.041; aOR 1.792, 95% CI 1.378~2.330) and leflunomide (aOR 1.525, 95% CI 1.196~1.944; aOR 1.740, 95% CI 1.372~2.207) were associated with an increased risk of AMI.

Conclusion: The study demonstrates a significant association between the current and long-term use of glucocorticoids and leflunomide and an increased risk of AMI in patients with RA. These findings underscore the importance of careful consideration of cardiovascular risks when selecting anti-rheumatic agents for RA treatment.

Macrophage activation syndrome (MAS) is a rare but potentially life-threatening complication of Kawasaki disease (KD). In Korea, many studies on KD have been reported, but there are only a few studies on MAS complicating KD (MAS-KD). This study was conducted to provide the characteristics of MAS-KD patients in Korea through a literature review. A total of 23 Korean patients with MAS-KD from 10 papers were included in this study. All MAS-KD patients met the hemophagocytic lymphohistiocytosis (HLH)-2004 criteria and/or the 2016 MAS criteria. The incidence of MAS-KD in Korean children was 0.8%~1.1%, which is relatively low compared to North America (1.9%). MAS-KD patients had lower rates of KD-related features and higher rates of incomplete KD, coronary artery abnormalities, and intravenous immunoglobulin resistance than patients with KD without MAS. Notable laboratory abnormalities in MAS-KD include anemia, neutropenia, thrombocytopenia, hypoalbuminemia, increased hepatic transaminase levels, and hyperferritinemia. For treatment of MAS-KD, the HLH-2004 protocol (i.e., 40 weeks of complex chemotherapy) was applied to 15 patients (65%), which is a significantly greater than those treated with this protocol in other countries (35%). Two patients (9%) died during the HLH-2004 protocol. In actual practice, MAS may be underrecognized in patients with KD. Clinical suspicion is paramount for early diagnosis and timely treatment.