{"title":"Pathogenesis of systemic sclerosis: an integrative review of recent advances.","authors":"Ha-Hee Son, Su-Jin Moon","doi":"10.4078/jrd.2024.0129","DOIUrl":"10.4078/jrd.2024.0129","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"32 2","pages":"89-104"},"PeriodicalIF":2.2,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11931279/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712158","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yongbum Kim, Nayeon Choi, Ji-Hui Shin, Sungsin Jo, Bora Nam, Tae-Hwan Kim
{"title":"Factors associated with anti-drug antibody production in ankylosing spondylitis patients treated with the infliximab biosimilar CT-P13.","authors":"Yongbum Kim, Nayeon Choi, Ji-Hui Shin, Sungsin Jo, Bora Nam, Tae-Hwan Kim","doi":"10.4078/jrd.2024.0114","DOIUrl":"10.4078/jrd.2024.0114","url":null,"abstract":"<p><strong>Objective: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"32 2","pages":"136-144"},"PeriodicalIF":2.2,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11931275/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712221","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Deep learning analysis for rheumatologic imaging: current trends, future directions, and the role of human.","authors":"Jucheol Moon, Pratik Jadhav, Sangtae Choi","doi":"10.4078/jrd.2024.0128","DOIUrl":"10.4078/jrd.2024.0128","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"32 2","pages":"73-88"},"PeriodicalIF":2.2,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11931281/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712215","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Soo Min Ahn, Seonok Kim, Ye-Jee Kim, Seokchan Hong, Chang-Keun Lee, Bin Yoo, Ji Seon Oh, Yong-Gil Kim
{"title":"Risk of acute myocardial infarction associated with anti-rheumatic agents in patients with rheumatoid arthritis: a nationwide population-based case-control study.","authors":"Soo Min Ahn, Seonok Kim, Ye-Jee Kim, Seokchan Hong, Chang-Keun Lee, Bin Yoo, Ji Seon Oh, Yong-Gil Kim","doi":"10.4078/jrd.2024.0104","DOIUrl":"10.4078/jrd.2024.0104","url":null,"abstract":"<p><strong>Objective: </strong>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).</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"32 2","pages":"113-121"},"PeriodicalIF":2.2,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11931272/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712161","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Macrophage activation syndrome in Kawasaki disease: a literature review of Korean studies.","authors":"Dae Chul Jeong, Soo-Young Lee","doi":"10.4078/jrd.2024.0118","DOIUrl":"10.4078/jrd.2024.0118","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"32 2","pages":"105-112"},"PeriodicalIF":2.2,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11931280/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712153","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Dionicio Angel Galarza-Delgado, Valeria Gonzalez-Gonzalez, Natalia Guajardo-Jauregui, Jesus Alberto Cardenas-de la Garza, Rosa Icela Arvizu-Rivera, Maria Fernanda Elizondo-Benitez, Andrea Lizbeth Guajardo-Aldaco, Jose Ramon Azpiri-Lopez, Iris Jazmin Colunga-Pedraza
{"title":"Evaluation of novel cardiovascular risk calculators in patients with rheumatoid arthritis.","authors":"Dionicio Angel Galarza-Delgado, Valeria Gonzalez-Gonzalez, Natalia Guajardo-Jauregui, Jesus Alberto Cardenas-de la Garza, Rosa Icela Arvizu-Rivera, Maria Fernanda Elizondo-Benitez, Andrea Lizbeth Guajardo-Aldaco, Jose Ramon Azpiri-Lopez, Iris Jazmin Colunga-Pedraza","doi":"10.4078/jrd.2024.0093","DOIUrl":"10.4078/jrd.2024.0093","url":null,"abstract":"","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"32 2","pages":"145-147"},"PeriodicalIF":2.2,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11931277/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712219","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Circulating VEGF levels and genetic polymorphisms in Behçet's disease: a meta-analysis.","authors":"Young Ho Lee, Gwan Gyu Song","doi":"10.4078/jrd.2024.0103","DOIUrl":"10.4078/jrd.2024.0103","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to explore the relationship between circulating vascular endothelial growth factor (VEGF) levels and Behçet's disease (BD), as well as to examine the association between VEGF gene polymorphisms and BD.</p><p><strong>Methods: </strong>We conducted a comprehensive search of the MEDLINE, Embase, and Web of Science databases to identify relevant research articles. A meta-analysis was performed to compare serum or plasma VEGF levels in BD patients with those in control groups. Additionally, we evaluated the potential associations between BD susceptibility and specific VEGF polymorphisms, namely -634 C/G, +936 C/T, and the 18 bp insertion/deletion (I/D) at -2549.</p><p><strong>Results: </strong>The analysis included 15 studies with a total of 1,020 BD patients and 1,031 controls. BD patients exhibited significantly higher circulating VEGF levels compared to controls (standardized mean difference [SMD]=1.726, 95% confidence interval [CI]=1.030~2.421, p<0.001). Elevated VEGF levels were noted among BD patients from European and Arab populations. Subgroup analysis further confirmed the increase in VEGF levels across different data types and sample sizes. Patients with active BD had higher VEGF levels than those with inactive BD (SMD=0.635, 95% CI=0.092~1.177, p=0.022). However, no significant association was found between BD and the VEGF -634 C allele (odds ratio=1.023, 95% CI=0.707~1.481, p=0.904). Similarly, no association was detected between BD and the VEGF +936 C/T or 18 bp I/D at -2549 polymorphisms.</p><p><strong>Conclusion: </strong>Our meta-analysis showed a strong association between elevated circulating VEGF levels and BD. However, the VEGF polymorphisms examined in this study do not appear to be associated with susceptibility to BD.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"32 2","pages":"122-129"},"PeriodicalIF":2.2,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11931276/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712213","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Navigating complexity through \"the groove\": eosinophilic fasciitis in the context of diabetes and lichen planus.","authors":"Sukdev Manna","doi":"10.4078/jrd.2024.0135","DOIUrl":"10.4078/jrd.2024.0135","url":null,"abstract":"","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"32 2","pages":"148-149"},"PeriodicalIF":2.2,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11931274/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712155","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Rheumatoid arthritis and pain management: the significance of psychological factors.","authors":"Hyun-Ok Kim","doi":"10.4078/jrd.2025.0022","DOIUrl":"10.4078/jrd.2025.0022","url":null,"abstract":"","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"32 2","pages":"71-72"},"PeriodicalIF":2.2,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11931273/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712159","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Roshan Subedi, Afrah Misbah, Adnan Al Najada, Anthony James Ocon
{"title":"Evaluation of 14-3-3eta protein as a diagnostic biomarker in the initial assessment of inflammatory arthritis.","authors":"Roshan Subedi, Afrah Misbah, Adnan Al Najada, Anthony James Ocon","doi":"10.4078/jrd.2024.0110","DOIUrl":"10.4078/jrd.2024.0110","url":null,"abstract":"<p><strong>Objective: </strong>Serum 14-3-3eta are novel biomarkers of rheumatoid arthritis (RA). It is not clear whether 14-3-3eta may be present in other forms of inflammatory arthritis (IA). We evaluated the presence of 14-3-3eta as a diagnostic biomarker in the evaluation IA.</p><p><strong>Methods: </strong>A retrospective cohort study of adult patients who were evaluated for IA by a rheumatologist with a result for the lab test of 14-3-3eta was conducted.</p><p><strong>Results: </strong>Of 280 included patients, 30% were diagnosed with RA, 11% with psoriatic arthritis (PsA), and 59% with another condition. Twenty-four (9%) patients had positive results for 14-3-3eta. Fifty-two percent of positive patients were diagnosed with RA, with 48% having another diagnosis including axial spondyloarthritis, gout, Sjögren's, undifferentiated IA, diabetic cheiroarthropathy, prostate cancer with bone metastasis, osteoarthritis, unspecified arthralgia. No patients with PsA had a positive value. RA patients had a higher value for 14-3-3eta compared to non-RA (5.44 [1.56~9.31] vs. 0.69 [0.40~0.98] ng/mL, p=0.03, square brackets are 95% confidence interval values). The mean value for the 14-3-3eta in seropositive RA trended higher than seronegative (8.0 [2.3~13.7] vs. 1.4 [0.4~2.4] ng/mL, p=0.06). In the RA cohort, elevated 14-3-3eta was associated with elevated erythrocyte sedimentation rate (odd ratio=6.62 [1.24~47.09], p<0.04), but not other variables.</p><p><strong>Conclusion: </strong>14-3-3eta may aid as a diagnostic biomarker of RA. However, it is not specific for RA, especially at low positive levels, and may be positive in other forms of IA. Ideal cutoff values need to be established for RA and non-RA conditions. It was not found in PsA.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":"32 2","pages":"130-135"},"PeriodicalIF":2.2,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11931278/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712217","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}