Pediatric Rheumatology最新文献

{"title":"Prognostic factors for disease progression and mortality of childhood-onset lupus nephritis in the Philippines: a retrospective cohort study in a tertiary hospital.","authors":"Carla Marie L Asis, Cherica A Tee, Lourdes Paula R Resontoc","doi":"10.1186/s12969-025-01128-w","DOIUrl":"10.1186/s12969-025-01128-w","url":null,"abstract":"<p><strong>Background: </strong>Lupus nephritis (LN) is a significant cause of morbidity and mortality in pediatric systemic lupus erythematosus (SLE), with more severe disease seen in childhood-onset cases. In low-to-middle-income countries like the Philippines, financial barriers and healthcare limitations exacerbate poor outcomes. This study aims to describe the clinical features, prognostic factors, and outcomes of childhood-onset LN at the Philippine General Hospital.</p><p><strong>Methods: </strong>A retrospective cohort study was conducted on patients under 19 years diagnosed with LN from January 2014 to December 2021. Clinical, laboratory, treatment, and outcome data were extracted from medical records. Survival was estimated using the Kaplan-Meier method. Cox proportional hazards regression and logistic regression were used to identify predictors of mortality and progression to chronic kidney disease 5 (CKD 5), respectively.</p><p><strong>Results: </strong>A total of 128 patients were included, with a mean age of 14.4 years at LN diagnosis and a female-to-male ratio of 13.2:1. Only 16% underwent kidney biopsy, mainly due to financial constraints. Treatment delays and poor adherence occurred in 60% and 38% of patients, respectively, largely due to financial hardship and limited healthcare access. The median follow-up was 2.2 years (range, 0.0-8.8 years). Nephrotic range proteinuria (hazard ratio [HR] 2.91), hypertension at diagnosis (odds ratio [OR] 5.57), and failure to achieve early partial response or complete remission (HR 3.69) were significant predictors of poor health outcomes. Twenty five patients (19.5%) died during the observation period with infection as the leading cause of mortality.</p><p><strong>Conclusions: </strong>Childhood-onset LN remains associated with high morbidity and mortality in the Philippines, highlighting the urgent need for early diagnosis, expanded diagnostic access, infection risk mitigation, early therapeutic response monitoring, and strategies to enhance treatment adherence to improve outcomes.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"86"},"PeriodicalIF":2.3,"publicationDate":"2025-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12318420/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144769357","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}

{"title":"Preliminary validation of a web-based MRI scoring system for children with chronic nonbacterial osteomyelitis (ChRonic nonbacterial Osteomyelitis Magnetic Resonance Imaging Scoring: CROMRIS).","authors":"Farzana Nuruzzaman, T Shawn Sato, Jennifer Stimec, Ramesh S Iyer, Andrew Carbert, Joel Paschke, Lauren Potts, Meinrad Beer, Ming Huang, Johanna Monsalve, Anh-Vu Ngo, Mahesh Thapa, Xiaoyue Zhang, Walter P Maksymowych, Polly J Ferguson, Yongdong Zhao","doi":"10.1186/s12969-025-01135-x","DOIUrl":"10.1186/s12969-025-01135-x","url":null,"abstract":"<p><strong>Background: </strong>The ChRonic nonbacterial Osteomyelitis Magnetic Resonance Imaging Scoring (CROMRIS) tool was developed to assess specific characteristics of bone and soft tissue inflammation on MR images of patients with CNO; however, this tool was labor intensive to utilize. We aimed (1) to refine and adapt this scoring method, (2) to assess the usability of this web-based CROMRIS system among radiologists and (3) to evaluate the absolute agreement of the components and summary CROMRIS scores at each body site, and the interrater reliability.</p><p><strong>Methods: </strong>We used a qualitative, user-centered design approach involving software developers, rheumatologists, radiologists, and a patient artist to adapt the paper-based scoring system to a web-based prototype that was further refined by monthly meetings between the group members. A clickable-schematic-based CROMRIS system was developed to include all body regions: head (skull/mandible), spine, torso (clavicle, sternum, and ribs), pelvis, hands, feet, arms, and legs. Readers scored individual bone units to indicate the presence of bone marrow hyperintensity on STIR images (score 0-1), soft tissue/periosteal hyperintensity of surrounding tissue (score 0-1), and bony expansion (score 0-1), and quantified the signal size of the CNO lesion (scores 1-3 defined as < 25%, 25-50%, or > 50% of the estimated volume, respectively). The sum of these parameters for lesions detected on fluid-sensitive sequences was the CROMIS Activity Index (maximum score 720). Feedback for usability was reported with descriptive content analysis and continuous variables as means and categorical variables as percentages. Interrater reliability was assessed by free-marginal kappa (k) statistics and the intraclass correlation coefficient (ICC).</p><p><strong>Results: </strong>The mean system usability score increased from 64.5 (below average) to 75 (above average) after user feedback. Interrater reliability for the CROMRIS Activity Index was excellent for clavicle, tibia, cervical and lumbar spines (> 0.9) and good to moderate for the remainder of the body regions. The mean kappa of each category of bones was > 0.6 demonstrating substantial interrater reliability among radiologists for the bone sites most affected by CNO, namely the long bones and clavicle.</p><p><strong>Conclusion: </strong>The web-based CROMRIS portal developed was usable and showed substantial-moderate agreement in the total CROMRIS Activity Index total scores among experienced radiologists after self-guided learning of the atlas and video. This tool can potentially be used in future clinical trials after calibration.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"85"},"PeriodicalIF":2.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12317626/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144765795","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}

{"title":"Feasibility of applying infrared thermal imaging for home monitoring of arthritis in children.","authors":"Stephen Wong, Nivrutti Bhide, Erin Balay-Dustrude, Erin Sullivan, Joshua Scheck, Ian Muse, Kevin Cain, Debosmita Biswas, Savannah C Partridge, Yongdong Zhao","doi":"10.1186/s12969-025-01096-1","DOIUrl":"10.1186/s12969-025-01096-1","url":null,"abstract":"<p><strong>Background: </strong>Telemedicine has improved access to pediatric rheumatology care. A disadvantage to using virtual modality for evaluation of children with arthritis is the lack of an in-person, hands-on physical exam. Thermal imaging has been studied in the clinical setting with promising results. This study aims to determine the feasibility of procuring at-home thermal imaging, measuring the variability of in-home skin temperature measurements over three consecutive days, and to compare these measurements at home to ones obtained in the clinic setting.</p><p><strong>Methods: </strong>Children with knee pain and/or swelling for a week or longer were enrolled and imaged with a smartphone-attached FLIR ONE PRO and Fluke handheld cameras followed by imaging with a FLIR camera at home for 3 consecutive days. Joint exam performed in the office was used as gold standard for joint assessment. A previously validated metric of temperature after within-limb calibration (TAWiC), defined as the temperature differences between the knee joint and ipsilateral mid-tibia, was used for all imaging studies.</p><p><strong>Results: </strong>Fifty-three patients were enrolled and thirty-eight completed the imaging acquisition at home with analyzable images. When evaluating images of the knee and mid-tibia regions, images collected at home compared to in-office demonstrated consistently lower absolute temperatures. However, the calibrated temperatures (TAWiC) of the anterior and lateral views of the knee showed mild to moderate correlation across 3 days between home-acquired images and office-acquired images (r = 0.58, 0.26, 0.24 and r = 0.36, 0.41, 0.42, respectively). The sensitivity and specificity of detecting arthritis of the knee using TAWiC adjustments from previously defined thresholds were similar regardless of the setting of image acquisition (0.44 and 0.79).</p><p><strong>Conclusions: </strong>This study demonstrates the feasibility of applying TAWiC for arthritis detection through a smartphone-based infrared thermal camera operated by families at home. Further investigation on a larger scale is needed prior to implementation of this process in the telemedicine setting.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"84"},"PeriodicalIF":2.3,"publicationDate":"2025-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12312333/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144762275","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}

{"title":"Consanguinity and rare monogenic systemic autoinflammatory disorders: implications for prevalence and genetic variability.","authors":"Alhanouf AlSaleem, Sulaiman M Al-Mayouf","doi":"10.1186/s12969-025-01133-z","DOIUrl":"10.1186/s12969-025-01133-z","url":null,"abstract":"","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"83"},"PeriodicalIF":2.3,"publicationDate":"2025-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12312363/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144755138","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}

{"title":"Expanding the phenotypic and genetic spectrum of ORAS with novel homozygous variant in OTULIN gene: a case series and literature review.","authors":"Alhanouf AlSaleem, Abdulrahman Assiri, Rayyan Alfadda, Suliman Al-Jumaah, Sulaiman M Al-Mayouf","doi":"10.1186/s12969-025-01129-9","DOIUrl":"10.1186/s12969-025-01129-9","url":null,"abstract":"","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"81"},"PeriodicalIF":2.3,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12309049/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144745887","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}

{"title":"A tale of two regions: comparing clinical features and outcomes in pediatric enthesitis-related arthritis.","authors":"Fatma Gül Demirkan, Vafa Guliyeva, Özlem Akgün, Hanene Lassoued Ferjani, Dorra Ben Nessib, Kawther Maatallah, Dhia Kaffel, Wafa Hamdi, Nuray Aktay Ayaz","doi":"10.1186/s12969-025-01095-2","DOIUrl":"10.1186/s12969-025-01095-2","url":null,"abstract":"<p><strong>Background: </strong>Enthesitis-related arthritis (ERA) may exhibit a distinct disease spectrum on the basis of ethnic origin. The pediatric rheumatology teams from the Istanbul Medical Faculty and Tunisia Kassab Institute engaged in collaboration via the Second Sister Hospital Initiative of the European Society of Pediatric Rheumatology (PReS) to investigate the clinical characteristics and outcomes of children with ERA.</p><p><strong>Methods: </strong>The medical records of patients with the diagnosis of ERA were reviewed retrospectively. The Juvenile Spondyloarthritis Disease Activity Index (JSpADA) was the tool for assessing disease activity. In addition to clinical and laboratory findings, treatments and disease outcomes were compared.</p><p><strong>Results: </strong>A total of 94 children with ERA were enrolled (45 Tunisian, 49 Turkish). Sex and age at disease onset were similar between the groups. Heel pain (8.8% vs. 61.2% for Tunisia vs. Türkiye, p = 0.03) and enthesitis (40% vs. 69.3% for Tunisia vs. Türkiye, p = 0.03, p = 0.8) were more common in Turkish children. Conversely, the rates of sacroiliac tenderness, suggesting clinical sacroiliitis (91.1% vs. 55.1% for Tunisia vs. Türkiye), and axial disease (97.8% vs. 55.1% for Tunisia vs. Türkiye) were significantly greater in Tunisian children (p = 0.002 and p < 0.001, respectively). Overall, 45.7% of the cohort was HLA-B27 positive, including 32% of Turkish patients and 60% of Tunisian patients (p < 0.001). HLA-B27 positivity did not influence age at disease onset (p = 0.45) but was associated with a longer diagnostic delay of the disease (p < 0.001). Nearly half of the Turkish children received biologics during the disease course, whereas only 8.9% of the Tunisian children did. While the median JSpADA scores at disease onset were similar between the groups, Turkish patients had significantly lower scores at the last visit than Tunisian patients did (p < 0.001).</p><p><strong>Conclusions: </strong>This study highlights notable differences in the clinical features and outcomes of ERA among Turkish and Tunisian children, emphasizing the potential influence of ethnic and regional factors on disease presentation and management. Variations in HLA-B27 positivity and treatment approaches, including the use of biologics, further underscore the need for tailored strategies in managing ERA across diverse populations.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"82"},"PeriodicalIF":2.3,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12309154/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144745886","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}

{"title":"Risk prediction models for renal injury in children with IgA vasculitis: a systematic review and meta-analysis.","authors":"Jianrong Liao, Xuqiong Tan, Fengbi Jiang, Lin Zhu, Ping Zhou","doi":"10.1186/s12969-025-01120-4","DOIUrl":"10.1186/s12969-025-01120-4","url":null,"abstract":"<p><strong>Aims: </strong>The goal of this systematic review and meta-analysis was to provide references for future researchers on how to develop and implement predictive models for renal injury in paediatric IgA vasculitis (IgAV).</p><p><strong>Design: </strong>Systematic review and meta-analysis of observational studies.</p><p><strong>Methods: </strong>We systematically searched databases including China National Knowledge Infrastructure (CNKI), Wanfang Database, China Science and Technology Journal Database (VIP), SinoMed, PubMed, Web of Science, Cochrane Library, and Embase for studies on the construction of predictive models for renal injury in children with IgAV, up until 24 November 2024. Two researchers independently screened the studies, extracted data, and assessed bias risk via the Prediction Model Risk of Bias Assessment Tool (PROBAST). STATA 16.0 software was used to conduct meta-analysis of the area under the curve (AUC) values of the models.</p><p><strong>Results: </strong>A total of 1,157 studies were retrieved. And 11 studies met the inclusion criteria. The sample sizes ranged from 155 to 583, with a renal injury incidence of 26.7-63.8%. The most common predictors included age, recurrent or persistent purpura, immunoglobulin A (IgA), D-dimer, and serum albumin (ALB). The included studies showed good overall applicability, however all were highly biased, mainly because they used inadequate data sources and reported poorly in the area analyzed. The pooled AUC of the five models was 0.86 (95% CI: 0.80-0.92), demonstrating good predictive power.</p><p><strong>Conclusion: </strong>In spite of the fact that the renal injury prediction model was found to be somewhat predictive in children with IgAV, all of them had a high risk of bias according to the PROBAST checklist. For these predictive tools to be more robust and clinically applicable, new models with larger sample sizes, rigorous designs, and external validation should be developed in the future.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"80"},"PeriodicalIF":2.3,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12305928/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144735485","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}

{"title":"The utility of miR-16, miR-146a and miR-155 in serum and urine for juvenile idiopathic arthritis diagnostics and monitoring.","authors":"Ausra Snipaitiene, Kristina Snipaitiene, Andzelika Slegeryte, Benita Buragaite-Staponkiene, Asta Baranauskaite, Sonata Jarmalaite, Lina Jankauskaite","doi":"10.1186/s12969-025-01136-w","DOIUrl":"10.1186/s12969-025-01136-w","url":null,"abstract":"<p><strong>Background: </strong>Biomarker search for juvenile idiopathic arthritis (JIA) diagnosis and monitoring remain the focus of research worldwide. Several microRNAs (miRNAs) have been identified as relevant in different rheumatic conditions; however, studies in JIA remain limited. Our study aimed to explore the potential of serum and urine-derived miRNAs for JIA diagnostics and longitudinal JIA monitoring.</p><p><strong>Methods: </strong>In this single-center, prospective study, three selected miRNAs (miR-16, -146a and -155) were tested in serial serum and urine samples collected from 31 JIA patients and 22 healthy controls (HC) via quantitative reverse transcription polymerase chain reaction (RT‒qPCR). The diagnostic performance of variables for distinguishing JIA patients from HCs was assessed by determining the area under the receiver operating characteristic (ROC) curve (AUC). The prediction of remission was evaluated using Cox regression and Kaplan-Meier analyses. A p-value < 0.05 was considered statistically significant.</p><p><strong>Results: </strong>Lower miR-16 and higher miR-155 levels were detected in serum of JIA patients' vs. HC (p < 0.01), whereas the level of miR-146a was lower in urine of JIA patients (p = 0.032). In ROC analysis, miR-16 and miR-155 distinguished JIA patients from HC when analyzed in serum (AUC 0.81, 95% CI 0.70-0.93, p < 0.001 and AUC 0.73, 95% CI 0.59-0.87, p = 0.005, respectively), and miR-146a- in urine (AUC 0.68, 95% CI 0.53-0.82, p = 0.030). During 12 months follow-up period increasing miR-16 (p = 0.021) and decreasing miR-155 (p = 0.009) levels were observed in serum samples. Kaplan-Meier survival analysis revealed that a high level of miR-146a in serum significantly predicts JIA remission (HR = 2.2, 95% CI 0.7-6.9, p = 0.040).</p><p><strong>Conclusions: </strong>This study highlights the utility of miRNAs in JIA diagnosis, monitoring and prognosis and demonstrates the feasibility of using urine as a noninvasive source of miRNAs in children with non-systemic JIA.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"79"},"PeriodicalIF":2.3,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12302888/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144735486","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}

{"title":"Mevalonate kinase deficiency: genetic and clinical characteristics of a Chinese pediatric cohort.","authors":"Chenchen Guan, Wenjie Wang, Qinhua Zhou, Jinqiao Sun, Lipin Liu, Luyao Liu, Bijun Sun, Jia Hou, Xiaochuan Wang","doi":"10.1186/s12969-025-01131-1","DOIUrl":"10.1186/s12969-025-01131-1","url":null,"abstract":"<p><strong>Background: </strong>Mevalonate kinase deficiency (MKD) is a rare autoinflammatory disease, and mevalonic aciduria (MA) is a severe phenotype of MKD. The present study reports the characteristics of MKD and four novel mutations in the mevalonate kinase (MVK) gene in a Chinese cohort.</p><p><strong>Method: </strong>A retrospective study was conducted on patients diagnosed with MKD from July 2013 to December 2024. The clinical, immunological, and follow-up data were collected from electronic medical records. Next-generation sequencing and Sanger sequencing were performed to identify gene mutations. A literature review was performed on MKD patients to further investigate the associations between genotype and phenotype.</p><p><strong>Results: </strong>Eleven MKD patients were enrolled from a Chinese cohort of prolonged and recurrent fever of unknown origin. Ten patients were classified as having hyperimmunoglobulin D syndrome (HIDS), and one patient was classified as having MA. The median follow-up duration was 5 years (IQR: 1.5-6 years). Recurrent fever and gastrointestinal symptoms were the most common symptoms. Anemia was observed in 8 of the 11 patients, including one patient with severe hematological complications. Growth restriction (5/11 patients) and developmental delay (4/11 patients) were also observed. IgD levels were measured in ten patients, and the median IgD level was 85.23 µg/ml (IQR: 18.74-385.19 µg/ml). Four novel mutation sites in the MVK gene were discovered: c.78G > A, c.463G > A, c.1076C > T and c.1088G > A. Etanercept was the effective biological agent tested, leading to complete or partial remission in 5 of the 6 patients. A literature review of 20 MA patients suggested that homozygous MVK gene mutations are more frequently associated with MA. Moreover, MA patients with the homozygous A334T mutation present a milder phenotype, and those with the I268T homozygous mutation present a more severe phenotype.</p><p><strong>Conclusions: </strong>This study is the largest case series of MKD pediatric patients from China. Four new mutation sites in MVK were identified, further expanding the phenotypic and genotypic spectrum of MKD and emphasizing the significance of MVK mutation patterns in influencing disease severity.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"78"},"PeriodicalIF":2.3,"publicationDate":"2025-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12297814/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144735484","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}

{"title":"Disease activity at two consecutive registry visits and subsequent medication escalation for patients with juvenile idiopathic arthritis in the CARRA registry.","authors":"Melissa L Mannion, Monica S Aswani, K Ria Hearld, Emily A Smitherman, Livie Timmerman, Jeffrey R Curtis","doi":"10.1186/s12969-025-01130-2","DOIUrl":"10.1186/s12969-025-01130-2","url":null,"abstract":"<p><strong>Objective: </strong>To account for the chronic time course of juvenile idiopathic arthritis (JIA), we assessed medication changes by disease activity patterns across 2 sequential timepoints.</p><p><strong>Methods: </strong>Patients with non-systemic JIA enrolled in the Childhood Arthritis and Rheumatology Research Alliance Registry with complete clinical Juvenile Arthritis Disease Activity Scores (cJADAS) at 6 and 12-month registry visits were included. Disease activity was classified by cJADAS categories (inactive/minimal, moderate/high). The primary outcome was disease modifying anti-rheumatic drug (DMARD) escalation at the 12-month visit. We examined the association between cJADAS patterns and DMARD escalation.</p><p><strong>Results: </strong>The cJADAS patterns across paired visits for 2,956 patients with JIA were: 71% persistent inactive/minimal, 25% persistent moderate/high, 2% \"improving\", and 2% \"flaring\". Only 10% of patients had DMARD escalation at the 12-month visit, including only 15% of patients with persistent moderate/high disease activity. In multivariable logistic regression adjusting for sociodemographic and clinical variables, DMARD escalation at the 12-month visit was associated with \"flaring\" disease activity (odds ratio [OR] 2.62, 95% confidence interval [CI] 1.33-5.18), DMARD escalation between the 6- and 12-month visits (OR 1.86, 95% CI 1.40-2.49) and morning stiffness (> 60 min 4.98, 95% CI 3.00-8.27), while age 15-19 years were less likely to escalate (OR 0.61, 95% CI 0.38-0.97).</p><p><strong>Conclusion: </strong>In a large multicenter registry of US patients with JIA, DMARD escalation at the 12-month visit was uncommon overall, even for those with persistent moderate/high disease activity. Our findings suggest that DMARD escalation in this cohort did not align well with a treat to target approach using cJADAS thresholds.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"77"},"PeriodicalIF":2.3,"publicationDate":"2025-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12285171/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144692485","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}