Pediatric Rheumatology最新文献

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Endpoints and outcomes for localized scleroderma/morphea: a scoping literature review. 局部硬皮病/褥疮的终点和结果:范围界定文献综述。
IF 2.8 3区 医学
Pediatric Rheumatology Pub Date : 2024-08-21 DOI: 10.1186/s12969-024-01014-x
Alexy Hernandez, Leslie Zapata Leiva, Maria Mutka, Kathryn S Torok, Leila Ledbetter, Christina K Zigler
{"title":"Endpoints and outcomes for localized scleroderma/morphea: a scoping literature review.","authors":"Alexy Hernandez, Leslie Zapata Leiva, Maria Mutka, Kathryn S Torok, Leila Ledbetter, Christina K Zigler","doi":"10.1186/s12969-024-01014-x","DOIUrl":"10.1186/s12969-024-01014-x","url":null,"abstract":"<p><strong>Background: </strong>Current treatment for localized scleroderma (LS) has been shown to halt disease activity, but little is still known about patient experiences with these treatments, nor is there consensus about optimal measurement strategies for future clinical trials.</p><p><strong>Objective: </strong>Conduct a scoping review of the literature for the types of outcomes and measures (i.e. clinician-, patient-, and caregiver-reported) utilized in published treatment studies of LS.</p><p><strong>Methods: </strong>Online databases were searched for articles related to the evaluation of treatment efficacy in LS with a special focus on pediatrics.</p><p><strong>Results: </strong>Of the 168 studies, the most common outcomes used were cutaneous disease activity and damage measured via clinician-reported assessments. The most frequently cited measure was the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT). Few patient-reported outcome measures (PROMs) were used.</p><p><strong>Limitations: </strong>Some studies only vaguely reported the measures utilized, and the review yielded a low number of clinical trials.</p><p><strong>Conclusion: </strong>In addition to evaluating disease activity with clinician-reported measures, the field could obtain critical knowledge on the patient experience by including high-quality PROMs of symptoms and functioning. More clinical trials using a variety of outcomes and measures are necessary to determine the most suitable course of treatment for LS patients.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"77"},"PeriodicalIF":2.8,"publicationDate":"2024-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11337887/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142019515","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A novel scoring system based on sIL-2R for predicting IVIG resistance in Chinese children with KD. 基于 sIL-2R 的新型评分系统用于预测中国 KD 患儿对 IVIG 的耐药性。
IF 2.8 3区 医学
Pediatric Rheumatology Pub Date : 2024-08-18 DOI: 10.1186/s12969-024-01015-w
Yuan-Yuan Zeng, Su-Yue Zhu, Kang-Kang Xu, Lian-Fu Ji, Yu-Qi Wang, Yi Chen, Feng Chen, Shi-Wei Yang
{"title":"A novel scoring system based on sIL-2R for predicting IVIG resistance in Chinese children with KD.","authors":"Yuan-Yuan Zeng, Su-Yue Zhu, Kang-Kang Xu, Lian-Fu Ji, Yu-Qi Wang, Yi Chen, Feng Chen, Shi-Wei Yang","doi":"10.1186/s12969-024-01015-w","DOIUrl":"10.1186/s12969-024-01015-w","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to develop a novel scoring system utilizing circulating interleukin (IL) levels to predict resistance to intravenous immunoglobulin (IVIG) in Chinese patients with Kawasaki disease (KD). We further compared this scoring system against six previously established scoring methods to evaluate its predictive performance.</p><p><strong>Methods: </strong>A retrospective analysis was conducted on KD patients who were treated at the cardiovascular medical ward of our institution from January 2020 to December 2022. Six scoring systems (Egami, Formosa, Harada, Kobayashi, Lan and Yang) were analyzed, and a new scoring system was developed based on our data.</p><p><strong>Results: </strong>In our study, 521 KD patients were recruited, 42 of whom (8.06%) were identified as resistant to IVIG. Our study indicated that IVIG-resistant KD patients were at an increased risk for the development of coronary arterial lesions (CALs) (P = 0.001). The evaluation of IVIG resistance using various scoring systems revealed differing levels of sensitivity and specificity, as follows: Egami (38.10% and 88.52%), Formosa (95.24% and 41.13%), Harada (78.57% and 43.22%), Kobayashi (66.67% and 74.95%), Lan (66.67% and 73.49%), and Yang (69.05% and 77.24%). Our novel scoring system utilizing sIL-2R demonstrated the highest sensitivity and specificity of 69.29% and 83.91%, respectively, and calibration curves indicated a favorable predictive accuracy of the model.</p><p><strong>Conclusion: </strong>Our newly developed scoring system utilizing sIL-2R demonstrated superior predictive performance in identifying IVIG resistance among Chinese patients with KD.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"76"},"PeriodicalIF":2.8,"publicationDate":"2024-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11330593/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142001330","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Handwriting speed in juvenile idiopathic arthritis using the detailed assessment of speed of handwriting. 利用手写速度的详细评估,研究幼年特发性关节炎患者的手写速度。
IF 2.8 3区 医学
Pediatric Rheumatology Pub Date : 2024-08-15 DOI: 10.1186/s12969-024-01013-y
C A Marchak, S James, I Davidson, J Brown, K Houghton
{"title":"Handwriting speed in juvenile idiopathic arthritis using the detailed assessment of speed of handwriting.","authors":"C A Marchak, S James, I Davidson, J Brown, K Houghton","doi":"10.1186/s12969-024-01013-y","DOIUrl":"10.1186/s12969-024-01013-y","url":null,"abstract":"<p><strong>Background: </strong>Handwriting is a commonly reported functional limitation for children with juvenile idiopathic arthritis (JIA). The aim of this study was to evaluate handwriting in children with JIA.</p><p><strong>Findings: </strong>Twelve children (mean age 13.0 years, SD = 1.9; range 9.1 to 15.6 years) with JIA completed the Detailed Assessment of Speed of Handwriting (DASH). The presence of hand and wrist arthritis, grip strength, disability, pain, and quality of life (QOL) was also assessed. The mean DASH score was 34.5th percentile (SD = 22.5). Eight (75%) scored below the 50th centile. DASH scores were negatively associated with grip strength (r = -0.31).</p><p><strong>Conclusions: </strong>Handwriting difficulties are common in children with JIA. Handwriting assessment may be helpful to direct treatments, and advocate for support and accommodations in school.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"75"},"PeriodicalIF":2.8,"publicationDate":"2024-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11325831/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141989576","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Uncovering the hidden socioeconomic impact of juvenile idiopathic arthritis and paving the way for other rare childhood diseases: an international, cross-disciplinary, patient-centered approach (PAVE Consortium). 揭示幼年特发性关节炎隐藏的社会经济影响,为其他罕见儿童疾病铺平道路:一种国际性、跨学科、以患者为中心的方法(PAVE 联合会)。
IF 2.8 3区 医学
Pediatric Rheumatology Pub Date : 2024-08-09 DOI: 10.1186/s12969-024-01012-z
Deborah A Marshall, Brittany Gerber, Gillian R Currie, Jordi Antón, Lien De Somer, Michelle Dey, Tsipi Egert, Yona Egert, Lia Henan, Jens Klotsche, Laura Martinez Mifsut, Kirsten Minden, Christophe Normand, David Porte, Rotraud K Saurenmann, Joost F Swart, Yosef Uziel, Jennifer Wilson, Carine Wouters, Amit Ziv, Susanne M Benseler
{"title":"Uncovering the hidden socioeconomic impact of juvenile idiopathic arthritis and paving the way for other rare childhood diseases: an international, cross-disciplinary, patient-centered approach (PAVE Consortium).","authors":"Deborah A Marshall, Brittany Gerber, Gillian R Currie, Jordi Antón, Lien De Somer, Michelle Dey, Tsipi Egert, Yona Egert, Lia Henan, Jens Klotsche, Laura Martinez Mifsut, Kirsten Minden, Christophe Normand, David Porte, Rotraud K Saurenmann, Joost F Swart, Yosef Uziel, Jennifer Wilson, Carine Wouters, Amit Ziv, Susanne M Benseler","doi":"10.1186/s12969-024-01012-z","DOIUrl":"10.1186/s12969-024-01012-z","url":null,"abstract":"<p><strong>Background: </strong>Juvenile idiopathic arthritis (JIA) refers to a heterogeneous group of rheumatic conditions in children. Novel drugs have greatly improved disease outcomes; however, outcomes are impacted by limited awareness of the importance of early diagnosis and adequate treatment, and by differences in access across health systems. As a result, patients with JIA continue to be at risk for short- and long-term morbidity, as well as impacts on virtually all aspects of life of the child and family.</p><p><strong>Main body: </strong>Literature on the socioeconomic burden of JIA is largely focused on healthcare costs, and the impact of JIA on patients, families, and communities is not well understood. High quality evidence on the impact of JIA is needed to ensure that patients are receiving necessary support, timely diagnostics, and adequate treatment, and to inform decision making and resource allocation. This commentary introduces the European Joint Programme on Rare Diseases: Producing an Arthritis Value Framework with Economic Evidence: Paving the Way for Rare Childhood Diseases (PAVE) project, which will co-develop a patient-informed value framework to measure the impact of JIA on individuals and on society. With a patient-centered approach, fundamental to PAVE is the involvement of three patient advocacy organizations from Canada, Israel, and Europe, as active research partners co-designing all project phases and ensuring robust patient and family engagement. The framework will build on the findings of projects from six countries: Canada, Germany, Switzerland, Spain, Israel, and Belgium, exploring costs, outcomes (health, well-being), and unmet needs (uveitis, mental health, equity).</p><p><strong>Conclusion: </strong>This unique international collaboration will combine evidence on costs (from family to societal), outcomes (clinical, patient and family outcomes), and unmet needs, to co-design and build a framework with patients and families to capture the full impact of JIA. The framework will support the development of high-quality evidence, encompassing economic and clinical considerations, unmet needs, and patient perspectives, to inform equitable resource allocation, health system planning, and quality of care better aligned with the needs of children with JIA, their families, and communities. Knowledge gained from this novel approach may pave the way forward to be applied more broadly to other rare childhood diseases.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"74"},"PeriodicalIF":2.8,"publicationDate":"2024-08-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11312924/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141908375","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
CAR T cell therapy for refractory pediatric systemic lupus erythematosus: a new era of hope? 治疗难治性小儿系统性红斑狼疮的 CAR T 细胞疗法:希望的新时代?
IF 2.8 3区 医学
Pediatric Rheumatology Pub Date : 2024-08-08 DOI: 10.1186/s12969-024-00990-4
Ivana Stojkic, Lauren Harper, Samantha Coss, Mahmoud Kallash, Kyla Driest, Margaret Lamb, Stacy P Ardoin, Shoghik Akoghlanian
{"title":"CAR T cell therapy for refractory pediatric systemic lupus erythematosus: a new era of hope?","authors":"Ivana Stojkic, Lauren Harper, Samantha Coss, Mahmoud Kallash, Kyla Driest, Margaret Lamb, Stacy P Ardoin, Shoghik Akoghlanian","doi":"10.1186/s12969-024-00990-4","DOIUrl":"10.1186/s12969-024-00990-4","url":null,"abstract":"<p><p>Systemic lupus erythematosus (SLE) is a chronic autoimmune condition that can affect multiple organ systems and is heterogenous in its presentation and response to therapy. When diagnosed in childhood, SLE is associated with increased morbidity and mortality compared to adult SLE, often requiring substantial immunosuppression with the risk of significant side effects. There remains a significant unmet need for new therapies that can improve disease control and reduce glucocorticoid and other toxic medication exposure for patients with severe or refractory disease. The pathogenesis of SLE involves B cell dysregulation and autoantibody production, which are a hallmark of the disease. Currently approved B cell directed therapies often result in incomplete B cell depletion and may not target long-lived plasma cells responsible for SLE autoantibodies. It is hypothesized that by persistently eliminating both B cells and plasmablasts, CAR T therapy can halt autoimmunity and prevent organ damage in patient's refractory to current B cell-depleting treatments. Herein we summarize the current preclinical and clinical data utilizing CAR T cells for SLE and discuss the future of this treatment modality for lupus.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"72"},"PeriodicalIF":2.8,"publicationDate":"2024-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11308704/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141908373","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Increased vascular deposition of oxidized LDL in untreated juvenile dermatomyositis. 未经治疗的幼年皮肌炎血管中氧化低密度脂蛋白沉积增加。
IF 2.8 3区 医学
Pediatric Rheumatology Pub Date : 2024-08-08 DOI: 10.1186/s12969-024-01001-2
Jacob C Spitznagle, Akadia Kacha-Ochana, Joan M Cook-Mills, Gabrielle A Morgan, Lauren M Pachman
{"title":"Increased vascular deposition of oxidized LDL in untreated juvenile dermatomyositis.","authors":"Jacob C Spitznagle, Akadia Kacha-Ochana, Joan M Cook-Mills, Gabrielle A Morgan, Lauren M Pachman","doi":"10.1186/s12969-024-01001-2","DOIUrl":"10.1186/s12969-024-01001-2","url":null,"abstract":"<p><strong>Background: </strong>Juvenile dermatomyositis (JDM) is a systemic vasculopathy associated with metabolic derangements and possible increased risk for premature atherosclerosis. Oxidation of low-density lipoprotein (LDL) in the endothelium is an early step in atherosclerotic plaque formation. It is not known if oxidized LDL is altered in children with untreated JDM. The deposition of oxidized LDL in the vasculature of muscle biopsies (MBx) from patients with untreated JDM and pediatric controls was assessed.</p><p><strong>Findings: </strong>Frozen tissue sections of MRI-directed MBx from 20 female children with untreated JDM and 5 female controls were stained with DAPI and fluorescently labeled antibodies against von Willebrand factor (vWF) and LDL oxidized by copper (oxLDL). Blood vessels were identified by positive vWF staining, and total fluorescence of oxLDL within the vessel walls was measured. Children with untreated JDM had increased deposition of oxLDL in the walls of muscle vasculature compared to healthy children (difference in means ± SEM = 19.86 ± 8.195, p = 0.03). Within the JDM cohort, there was a trend towards increased oxLDL deposition with longer duration of untreated disease (r = 0.43, p = 0.06). There was no significant correlation found between oxLDL deposition and markers of acute JDM disease activity including disease activity scores or muscle enzymes.</p><p><strong>Conclusions: </strong>This study found increased deposition of oxLDL within blood vessels of children with untreated JDM supporting the concern that these children are at increased risk for premature atherosclerosis from chronic exposure to vascular oxLDL. This study highlights the importance of early diagnosis and treatment initiation to ameliorate cardiovascular damage.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"73"},"PeriodicalIF":2.8,"publicationDate":"2024-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11308466/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141908374","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Exploring the association between serum Vitamin D levels and the development of coronary artery lesions in Kawasaki disease - a systematic review. 探索血清维生素 D 水平与川崎病冠状动脉病变发展之间的关系--系统综述。
IF 2.8 3区 医学
Pediatric Rheumatology Pub Date : 2024-08-05 DOI: 10.1186/s12969-024-01010-1
Zahra Amirsardari, Fatemeh Amirsardari, Erfan Kohansal, Amir Ghaffari Jolfay, Maziar Gholampour Dehaki, Vahid Ziaee
{"title":"Exploring the association between serum Vitamin D levels and the development of coronary artery lesions in Kawasaki disease - a systematic review.","authors":"Zahra Amirsardari, Fatemeh Amirsardari, Erfan Kohansal, Amir Ghaffari Jolfay, Maziar Gholampour Dehaki, Vahid Ziaee","doi":"10.1186/s12969-024-01010-1","DOIUrl":"10.1186/s12969-024-01010-1","url":null,"abstract":"<p><strong>Background: </strong>Kawasaki Disease (KD) involves arterial inflammation, primarily affecting the coronary arteries and leading to coronary artery lesions. Recent advancements in understanding the immunomodulatory roles of vitamin D have prompted investigations into the potential correlation between serum vitamin D levels and the risk of coronary artery lesions (CAL) in KD. This review aims to explore this association.</p><p><strong>Methods: </strong>A systematic search utilizing relevant keywords related to Kawasaki disease and coronary artery lesions was conducted across four databases (PubMed, Embase, Scopus, and Web of Science). The quality of the incorporated studies was assessed utilizing the Newcastle-Ottawa Scale. The study protocol is registered in PROSPERO under the registry code CRD42024493204.</p><p><strong>Results: </strong>In a review of five studies involving 442 KD patients and 594 healthy controls, KD patients generally had lower serum vitamin D levels compared to controls, with mixed findings on the association with coronary artery lesions and IVIG resistance. While three studies supported lower vitamin D in KD, one showed no significant difference. Regarding CAL, one study found lower vitamin D, another found higher levels associated with CAL, and two found no significant difference.</p><p><strong>Conclusions: </strong>Overall, the evidence is inconclusive, but there's a trend suggesting potential benefits of sufficient vitamin D levels in Kawasaki disease rather than evidence refuting any association with clinical outcomes.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"71"},"PeriodicalIF":2.8,"publicationDate":"2024-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11299257/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141894893","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Assay for interferon gamma release as a novel marker in pediatric patients with systemic lupus erythematosus 将γ干扰素释放测定作为系统性红斑狼疮儿科患者的新型标记物
IF 2.5 3区 医学
Pediatric Rheumatology Pub Date : 2024-08-01 DOI: 10.1186/s12969-024-01008-9
Song Zhang, Xue Li, Huishan Chen, Xianfei Gao, Zhe Cai, Huasong Zeng
{"title":"Assay for interferon gamma release as a novel marker in pediatric patients with systemic lupus erythematosus","authors":"Song Zhang, Xue Li, Huishan Chen, Xianfei Gao, Zhe Cai, Huasong Zeng","doi":"10.1186/s12969-024-01008-9","DOIUrl":"https://doi.org/10.1186/s12969-024-01008-9","url":null,"abstract":"The interferon-gamma (IFN-γ) release assay (IGRA) is an important laboratory diagnosis for latent Mycobacterium tuberculosis (TB) infection. The TB-IGRA measures the release of IFN-γ from peripheral blood cells, who are exposed to TB antigen (Ag), mitogen (MT), or negative/nil control (NL) in vitro. While, an exceptional higher TB Ag-NL level will reflect an elevation of peripheral lymphocytes released IFN-γ in a same condition. Therefore, we found that the elevated levels of TB Ag-NL could become a new biomarker for the diagnosis and treatment of pediatric systemic lupus erythematosus (SLE) patients. We have analyzed the clinical data of 776 children who are underwent TB-IGRA testing in the Department of Allergy and Rheumatology of Guangzhou Women and Children’s Medical Center from 2018 to 2020. To investigate the association between TB Ag-NL and SLE, we have analyzed the clinical data of 47 SLE patients and TB Ag-NL testing results, and then evaluated the association between TB Ag-NL and SLE disease activity. The TB Ag-NL levels were significantly higher in patients with active SLE than those in inactive SLE (p = 0.0002). The TB Ag-NL levels were positively correlated with the SLE disease activity index (SLEDAI) and laboratory diagnosis parameters. The mean value of TB Ag-NL in SLE patients (0.04191 ± 0.07955, IU/mL) were significantly higher than those in patients with juvenile dermatomyositis (JDM) (0.0158 ± 0.0337, IU/mL, p = 0.036), juvenile idiopathic arthritis (JIA) (0.0162 ± 0.0388, IU/mL, p = 0.001), and healthy controls (HC) (0.0001 ± 0.0027, IU/mL, p = 0.0003). Therefore, the elevated TB Ag-NL levels could serve as a potential diagnostic biomarker of SLE, especially for the active SLE. The detection of IFN-γ release levels by the TB-IGRA may be useful to assess SLE disease activity in pediatric patients with active SLE. Spontaneous IFN-γ release is associated with Systemic lupus erythematosus in children. IFN-γ-releasing potential, as measured by the mycobacterium tuberculosis IFN-c release assay, associates with Systemic lupus erythematosus activity in children . IFN-γ release assays may offer a novel, blood-based approach to assessing SLE disease activity in children.","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"13 1","pages":""},"PeriodicalIF":2.5,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141867750","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Peripheral blood regulatory T cells and disease activity, quality of life, and outcomes in children with juvenile idiopathic arthritis 外周血调节性 T 细胞与幼年特发性关节炎患儿的疾病活动、生活质量和预后
IF 2.5 3区 医学
Pediatric Rheumatology Pub Date : 2024-08-01 DOI: 10.1186/s12969-024-01006-x
Neus Quilis, Pablo Mesa-del-Castillo Bermejo, Paula Boix, Oriol Juanola, Pilar Bernabeu, Rubén Francés, Mariano Andrés
{"title":"Peripheral blood regulatory T cells and disease activity, quality of life, and outcomes in children with juvenile idiopathic arthritis","authors":"Neus Quilis, Pablo Mesa-del-Castillo Bermejo, Paula Boix, Oriol Juanola, Pilar Bernabeu, Rubén Francés, Mariano Andrés","doi":"10.1186/s12969-024-01006-x","DOIUrl":"https://doi.org/10.1186/s12969-024-01006-x","url":null,"abstract":"To measure regulatory T cell (Treg) levels in the peripheral blood of children with juvenile idiopathic arthritis (JIA) and analyse the association of this measure with disease activity, quality of life, adjustment of treatment, and hospitalisation. We conducted a two-phase study (cross-sectional and prospective), including consecutive children with a JIA diagnosis according to ILAR criteria. Our independent variables were Tregs, Th1, Th2, and cytokines in peripheral blood, and our dependent variables in the cross-sectional phase were arthritis category, JIA activity, and patient-reported outcomes. To test associations, we used Spearman’s correlation coefficient and the Mann-Whitney U test. In the prospective phase, we explored the probability of treatment adjustment and hospitalisation for JIA during follow-up according to Tregs levels at baseline, using Cox proportional regression. Our sample included 87 participants (median age 11 years, 63.2% girls). Tregs were not associated with most variables of interest. However, we found that higher Tregs concentration was associated with lower erythrocyte sedimentation rate (ESR) and better subjective disease status and course, while higher IL-10 and TGF-β levels were associated with lower ESR, less pain, and better subjective disease status We found no association between Tregs and treatment adjustments or hospitalisation. Higher baseline Treg levels in the peripheral blood of children with JIA may be associated with reduced disease activity and better quality of life, though were not informative on the inflammatory progression on the follow-up. ","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"211 1","pages":""},"PeriodicalIF":2.5,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141867751","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Serological response after COVID-19 infection compared to vaccination against COVID-19 in children with autoimmune rheumatic diseases. 自身免疫性风湿病患儿感染 COVID-19 后的血清反应与接种 COVID-19 疫苗后的血清反应比较。
IF 2.8 3区 医学
Pediatric Rheumatology Pub Date : 2024-07-25 DOI: 10.1186/s12969-024-01003-0
Tjaša Šinkovec Savšek, Mojca Zajc Avramovič, Tadej Avčin, Miša Korva, Tatjana Avšič-Županc, Nataša Toplak
{"title":"Serological response after COVID-19 infection compared to vaccination against COVID-19 in children with autoimmune rheumatic diseases.","authors":"Tjaša Šinkovec Savšek, Mojca Zajc Avramovič, Tadej Avčin, Miša Korva, Tatjana Avšič-Županc, Nataša Toplak","doi":"10.1186/s12969-024-01003-0","DOIUrl":"10.1186/s12969-024-01003-0","url":null,"abstract":"<p><strong>Background: </strong>Paediatric patients with autoimmune rheumatic diseases (pARD) have a dysregulated immune system, so infections present a major threat to them. To prevent severe COVID-19 infections we aimed to vaccinate them as soon as possible. Studies have shown that the BNT162b2 vaccine is safe, effective, and immunogenic, however, in a short observation period, only.</p><p><strong>Methods: </strong>The main objective was to compare the serological response between three groups of pARD: after SARS-CoV-2 infection, after vaccination against COVID-19 with two doses of the BNT162b2 vaccine, and after experiencing both events. Data on demographics, diagnosis, therapy, and serology (anti-SARS-CoV-2 IgG/IgA) were collected from March 2020 to April 2022. For statistical analysis ANOVA, Mann-Whitney U test, Chi-square test and Fisher's exact test were applied. To compare adverse events (AE) after vaccination we included a control group of healthy adolescents.</p><p><strong>Results: </strong>We collected data from 115 pARD; from 92 after infection and 47 after vaccination. Twenty-four were included in both groups. Serological data were available for 47 pARD after infection, 25 after vaccination, and 21 after both events. Serological response was better after vaccination and after both events compared to after infection only. No effect of medication on the antibody levels was noted. The safety profile of the vaccine was good. Systemic AE after the first dose of the vaccine were more common in healthy adolescents compared to pARD. In the observation period of 41.3 weeks, 60% of vaccinated pARD did not experience a symptomatic COVID-19 infection.</p><p><strong>Conclusions: </strong>IgG and IgA anti-SARS-CoV-2 levels were higher after vaccination and after both events compared to after infection only. Six months after vaccination we observed an increase in antibody levels, suggesting that pARD had been exposed to SARS-CoV-2 but remained asymptomatic.</p><p><strong>Trial registration: </strong>The study was approved by the Medical Ethics Committee of the Republic of Slovenia (document number: 0120-485/2021/6).</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"68"},"PeriodicalIF":2.8,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11271209/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141762623","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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