Pediatric Rheumatology最新文献

{"title":"Effect of additional intravenous immunoglobulin for infliximab-refractory Kawasaki disease: a cohort study.","authors":"Satoki Hatano, Hiro Nakao, Hiroshi Masuda, Hiroshi Ono, Mitsuru Kubota, Akira Ishiguro","doi":"10.1186/s12969-025-01108-0","DOIUrl":"10.1186/s12969-025-01108-0","url":null,"abstract":"<p><strong>Background: </strong>Infliximab (IFX) is a reliable choice of treatment for intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients. Nationwide surveys in Japan demonstrated that additional treatment was still required for 20-30% of patients after IFX infusion. Additional IVIG was selected for 70% as the treatment for KD refractory to IFX. This study aimed to describe the therapeutic effect of IVIG after IFX for patients with KD refractory to IFX.</p><p><strong>Methods: </strong>A cohort study was conducted at a single center involving patients treated with additional IVIG for KD refractory to IFX between January 2016 and March 2023 (IVIG-after-IFX group). Additionally, KD patients resistant to the initial IVIG and who received a second IVIG in 2016 were included as a comparison group (second-IVIG group). We employed descriptive statistics and survival analysis to describe their clinical course information, including the time from initiation of the treatment to resolution of fever and the appearance of coronary artery lesions (CALs).</p><p><strong>Results: </strong>The analysis included 27 cases in the IVIG-after-IFX group. The additional IVIG-after-IFX was initiated on a median 11 days of illness (range 8-29). The median time until fever resolution was 1.0 day in the IVIG-after-IFX group and 1.0 day in the second-IVIG group (P = 0.783, HR 1.00; 95% CI 0.58-1.70). The fever resolved within 2.0 days after the initiation of the therapy in 78% (21/27) in the IVIG-after-IFX group and 68% (26/38) in the second-IVIG group. CALs were identified in 26% (7/27) before initiating IVIG-after-IFX, and 7% (2/27) showed new CALs after IVIG after IFX. Persistent CALs were observed in 19% (5/27) after 12 months after diagnosis.</p><p><strong>Conclusions: </strong>Additional IVIG for IFX-refractory KD may have a therapeutic effect comparable to that of the second IVIG for IVIG-resistant KD and be a hopeful therapeutic option for IFX-refractory KD. Treatment of IFX-refractory KD remains a challenge for us and requires further exploration, particularly regarding CAL prevention.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"49"},"PeriodicalIF":2.8,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12076848/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144046274","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":"FDG-PET/CT as a useful tool for disease activity assessment in large vessel vasculitis in childhood.","authors":"Tobias Krickau, Armin Atzinger, Joerg Juengert, Juergen Rech, Oliver Rompel, André Hoerning","doi":"10.1186/s12969-025-01091-6","DOIUrl":"https://doi.org/10.1186/s12969-025-01091-6","url":null,"abstract":"","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"48"},"PeriodicalIF":2.8,"publicationDate":"2025-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12065355/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144041171","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":"Factors that influence school attendance and participation in children and adolescents with juvenile idiopathic arthritis - a systematic review of the literature.","authors":"Tom Spillane, Kieran Murray","doi":"10.1186/s12969-025-01101-7","DOIUrl":"10.1186/s12969-025-01101-7","url":null,"abstract":"<p><strong>Introduction: </strong>Juvenile Idiopathic Arthritis (JIA) is an umbrella term for causes of inflammatory arthritis in children and adolescents. Symptoms include pain, stiffness and fatigue and this can have an impact on a child's ability to attend school or participate in school activities, such as physical education.</p><p><strong>Methods: </strong>Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) methodology, the research databases Pubmed, EMBASE and CINAHL were searched using key words related to JIA and school attendance and participation. Inclusion criteria were being 18 years old or under, having a diagnosis of JIA and examining JIA's impact on either school attendance or participation in a school activity. Included studies were examined for factors that affected attendance or participation.</p><p><strong>Results: </strong>Twelve research articles were included in the literature review. Six themes were identified as affecting attendance and participation. They were (1) Symptoms and treatment of JIA (2) Disability (3) Psychological symptoms (4) Disease activity and JIA subtype (5) Communication and school support and (6) Factors that increased participation.</p><p><strong>Conclusion: </strong>Children and adolescents with JIA have reduced levels of school attendance and often cannot participate fully in school life due to their medical condition. Targeted strategies may improve participation rates in this population. Further research is needed in this area to develop interventions and strategies to facilitate the optimal educational and school experience possible for this cohort.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"47"},"PeriodicalIF":2.8,"publicationDate":"2025-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12063317/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144060745","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":"Developing consensus outcome measures in juvenile systemic sclerosis: a global survey of pediatric rheumatologists and literature review.","authors":"Natalia Vasquez Canizares, Clare E Pain, Francesco Zulian, Amra Adrovic Yildiz, Simone Appenzeller, Edoardo Marrani, Emanuela Del Giudice, Antonella Petaccia, Francesca Tirelli, Gabriele Simonini, Mustafa Çakan, Marco Cattalini, Paulo Rogerio Julio, Kathryn Torok, Raju Khubchandani, Ozgur Kasapcopur, Lauren Robinson, Eslam Al-Abadi, Aybuke Gunalp, Meiping Lu, Hanna Lythgoe, Amanda Robinson, Betul Sozeri, Susan Shenoi, Emily Willis, Katherine Clarke, Rongjun Zheng, Biagio Castaldi, Valentina Leone, Valerio Maniscalco, Lucy Stead, Lusine Ambartsumyan, Franziska Rosser, Phuoc Duong, Aurelia Minuti, Suzanne C Li, Marinka Twilt","doi":"10.1186/s12969-025-01100-8","DOIUrl":"10.1186/s12969-025-01100-8","url":null,"abstract":"<p><strong>Background: </strong>Juvenile systemic sclerosis (JSSc) is a rare multisystemic disease with high morbidity and mortality rates. Treatment options remain limited, and there is a significant unmet need for effective therapies. This study aims to address this gap by investigating current JSSc management practices and identifying key outcome measures that can be used to inform the development of standardized assessment tools for future clinical trials.</p><p><strong>Methods: </strong>A web-based survey was distributed to pediatric rheumatologists to assess cardiopulmonary assessment standard of care practices and immunosuppressive treatment use in JSSc. Respondents were categorized by region (North America, Europe, Latin America, and Asia/Africa), and country income level. A scoping literature review was conducted using the PRISMA-SCR framework to identify outcome measures for six domains in SSc.</p><p><strong>Results: </strong>One hundred forty-one pediatric rheumatologists from 26 countries completed the survey. Significant variations in JSSc cardiopulmonary assessment practices across regions and income levels were noted. Respondents in North America and Europe reported using pulmonary function tests (PFTs) with diffusing capacity of the lungs for carbon monoxide (DLCO) more frequently than those in Latin America, or Asia/Africa (p < 0.001). The 6-min walk test (6MWT) was used less frequently by respondents in North America than other regions (p = 0.004). Use of oral corticosteroid and cyclophosphamide for treatment of JSSc varies significantly based on country income level, with higher usage in low- and middle-income nations. The scoping review identified 848 relevant articles for data extraction (ranging from 36 to 156 per domain) from a pool of 31,825 records, which were screened in multiple stages by 39 investigators.</p><p><strong>Conclusion: </strong>We found significant variability in JSSc assessment and treatment preferences, influenced by geography and income. This highlights the urgent need for international collaboration and standardized approaches in JSSc care.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"46"},"PeriodicalIF":2.8,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12046749/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144046269","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":"Digital health tools in juvenile idiopathic arthritis: a systematic literature review.","authors":"Jordi Anton, María Montoro, Estíbaliz Loza, Teresa Otón, Susan Ramirez, Diego Benavent","doi":"10.1186/s12969-025-01094-3","DOIUrl":"https://doi.org/10.1186/s12969-025-01094-3","url":null,"abstract":"<p><strong>Background: </strong>Nowadays, digital health technologies, including mobile apps, wearable technologies, social media, websites, electronic medical records, and artificial intelligence, are impacting disease management and outcomes. We aimed to analyse the characteristics and use of digital health tools in juvenile idiopathic arthritis (JIA).</p><p><strong>Methods: </strong>We conducted a systematic review (SR) to identify articles examining the characteristics, use, and outcomes (feasibility, usability, and effectiveness) of digital health tools in JIA patients. A sensitive search strategy was performed in Medline, Embase, and Cochrane databases until December 2022 (later updated to March 2024). Two reviewers independently selected the studies and collected the data, including study quality. A descriptive analysis was performed.</p><p><strong>Results: </strong>A total of 21 studies were included, one SR, six randomised controlled trials, four observational studies, four validation studies, one discovery and verification study, and five qualitative studies. Study quality was generally moderate. Most studies focused on patients with JIA (especially young people), but also on parents and health care professionals. Different digital health technologies were investigated, like websites, mobile apps, wearables, and telemedicine. The main objectives of the tools were self-management, symptom and quality of life monitoring, physical activity tracking, disease knowledge improvement, and medication monitoring. Different themes and contents were usually included in the same digital health tool, such as psychological health, lifestyle, intimacy, or shared decision-making. Tool development and validation processes were poorly or not at all described, and data regarding regulatory compliance, security, or privacy were scarce.</p><p><strong>Conclusions: </strong>There is significant variability in the type, characteristics, objectives, and contents of digital health tools for JIA. They still show limitations and gaps, thus highlighting the need for better critical assessment and reporting.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"45"},"PeriodicalIF":2.8,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12046958/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144055695","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":"Influenza vaccination in patients with juvenile idiopathic arthritis under different treatments: safety and immune response.","authors":"Adam Gyori, Arnold Nagy, Gabor Ottoffy, Tamas Decsi, Diana Simon, Timea Berki, Timea Dergez, David Kuti, Bernadett Mosdosi","doi":"10.1186/s12969-025-01099-y","DOIUrl":"https://doi.org/10.1186/s12969-025-01099-y","url":null,"abstract":"<p><strong>Background: </strong>Annual flu vaccination is recommended for children with rheumatic diseases. We investigated the cellular and humoral immune response and safety in pediatric patients that received inactivated influenza vaccines.</p><p><strong>Methods: </strong>This is a comparative study of in 41 children with juvenile idiopathic arthritis (JIA) receiving influenza vaccination while being treated with methotrexate (MTX) or biological therapy. The influenza vaccination was administered as a single dose of trivalent influenza vaccine (TIV). Serological tests to monitor seroconversion and seroprotection were performed at baseline and at 4 as well as 12 weeks after vaccination.</p><p><strong>Results: </strong>In all of the 41 children with JIA and the 22 healthy children seroconversion and seroprotection were observed for Influenza A. For Influenza B, no adequate seroconversion rates were not detected in any of the groups studied. No significant differences were observed in lymphocyte subpopulations when analysing time points and groups simultaneously. There were no relapses or cases of influenza infection after the vaccination. Our findings do not suggest non-specific immune activation following vaccination based on the distribution and quantity of the lymphocyte subsets that were investigated.</p><p><strong>Conclusion: </strong>The present study demonstrates adequate seroprotection rates against influenza A in immunosuppressed children with JIA. The trivalent vaccine had good immunogenicity and was safe to use in both JIA treatment groups.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"43"},"PeriodicalIF":2.8,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12023564/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144060043","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":"Imaging assessment of calcinosis in juvenile dermatomyositis: a narrative review.","authors":"Belina Y Yi, Jessica Perfetto, Evin Rothschild, Kelly Rouster-Stevens, Amanda Robinson, Kathryn Cook, Delaney D Ding, Andrea Eagle Child, Ovgu Kul Cinar, Barbara Limbach, Charalampia Papadopoulou, Lesley Ann Saketkoo, Adam Schiffenbauer, Heinrike Schmeling, Antonia Valenzuela, Susan Shenoi, Dawn M Wahezi","doi":"10.1186/s12969-025-01098-z","DOIUrl":"https://doi.org/10.1186/s12969-025-01098-z","url":null,"abstract":"<p><p>Calcinosis is a severe manifestation of juvenile and adult idiopathic inflammatory myopathies, which can lead to pain, limited range of motion, disfigurement, and infection. It is more common in juvenile idiopathic inflammatory myopathies, especially in juvenile dermatomyositis (JDM). Calcinosis can be visible on cutaneous surfaces, although can also present in muscles and internal organs, making a thorough assessment difficult without imaging modalities. In this narrative review, we discuss different imaging modalities used in evaluating JDM-associated calcinosis including X-ray, computed tomography (CT), magnetic resonance imaging (MRI), and ultrasound (US).</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"44"},"PeriodicalIF":2.8,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12032750/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144006162","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":"Endocrine dysfunction in patients with juvenile idiopathic arthritis.","authors":"Sayan Mukherjee, Abilash Krishnan Vijayakumaran, Mukesh Kumar Maurya, Nishant Gautam Kamble, Ankush Pm, Puneet Kumar, Wahid Ali, Mala Kumar, Saurabh Kumar, Pankti Mehta, T G Sundaram, Urmila Dhakad","doi":"10.1186/s12969-025-01058-7","DOIUrl":"https://doi.org/10.1186/s12969-025-01058-7","url":null,"abstract":"<p><strong>Objectives: </strong>To assess the prevalence of endocrine dysfunction in patients with JIA and identify potential contributory factors for growth and sexual development.</p><p><strong>Methods: </strong>A prospective observational study was conducted between July 2021 to January 2023, recruited 107 children of JIA fulfilling the revised ILAR classification criteria with disease duration > 6 months, attending Rheumatology department in KGMU, India. Demographic, clinical (anthropometric), and serological (including hormonal) evaluations were assessed at baseline. Growth velocity was recorded after one year. Mann-Whitney U test, chi-square test, and Fisher's exact t test were applied during statistical analysis.</p><p><strong>Results: </strong>107 JIA patients were enrolled with a M: F ratio of 2.06:1 (72 boys & 35 girls) with ERA being the most frequent subtype (51.4%). Mean age was 13 (± 4) years with a disease duration of 33 (± 24) months. Mean glucocorticoid intake was 2.17 (± 5.41) mg/day at baseline. 20.6% children were stunted, 22.4% were underweight and 25.2% had low BMI. Stunted children were more likely to have early onset (p = 0.015) & high GH level (p = 0.013). Underweight children had longer disease (p = 0.047) and more damage (p = 0.006). Children with weight z < -2 have high GH and low IGFBP3. Low BMI group had high disease activity, damage, and poor quality of life & functional state (p = < 0.01). Delayed puberty was noticed only in 2.8% of children. Girls with low Estradiol level had longer exposure to corticosteroids. Slower growth velocity was observed in 22.4% of children without any identifiable cause.</p><p><strong>Conclusion: </strong>One third of JIA patients experience growth and pubertal disturbances, primarily due to altered GH-IGF1 axis.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"41"},"PeriodicalIF":2.8,"publicationDate":"2025-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12023601/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144058678","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":"Incidence and reasons for biologic and targeted synthetic DMARD switching in juvenile idiopathic arthritis: a real- life stratified analysis.","authors":"Daniel Clemente, Leticia Leon, Juan Carlos Nieto-Gonzalez, Alina Lucica Boteanu, Laura Trives Folguera, Antía Asunción García-Fernández, Helena Amar Muñoz, Aliuska Palomeque, Juan Carlos López Robledillo, Lydia Abasolo","doi":"10.1186/s12969-025-01097-0","DOIUrl":"https://doi.org/10.1186/s12969-025-01097-0","url":null,"abstract":"<p><strong>Background: </strong>Switching biologic and targeted synthetic DMARDs (b/tsDMARD) is common in juvenile idiopathic arthritis (JIA) patients, though information about how this switching is done is scarce. This study aimed to determine the incidence rate, reasons for switching, and risk factors associated with switching due to inefficacy across different JIA subtypes.</p><p><strong>Methods: </strong>A multi-hospital electronic health record (EHR) registry was used to identify JIA patients prescribed ≥ 1 b/tsDMARD between 2000 and 2024. Patients were categorized into four JIA subgroups: oligoarticular, polyarticular, juvenile spondyloarthritis (JSpA), and systemic JIA. The primary outcomes were switching rates and switching due to inefficacy. Incidence rates (IR) were calculated per 100 patients-year. Cox multivariate regression analyses were run to assess the risk of b/tsDMARDs switching due to inefficacy, expressed as hazard ratio (HR) and 95% CI.</p><p><strong>Results: </strong>In our JIA registry, a total of 213 patients received a b/tsDMARD, with a total of 321 courses. The mean age at onset was 6.03 ± 4.44 years and 66.20% were females. The oligoarticular course group included 69 patients (32.39%), the polyarticular group 76 patients (35.68%), the JSpA group 43 patients (20.19%), and the systemic group 25 patients (11.74%). We found a total of 100 b/tsDMARD switches, with 32.05% of patients switched at least once. The systemic JIA group was more likely to swapping (p ≤ 0.001). Through the study period, the overall switching incidence rate was 7.32 [6.01-8.90] per 100 patients-year. In the stratified analysis across JIA groups, the systemic JIA group exhibited the highest incidence (IR:17.01 [11.20-25.84]). Regarding switching due to inefficacy, global incidence was 4.53 [3.53-5.82] and again systemic JIA was the group with the highest incidence (IR: 9.28 [5.27-16.34]). Still, the adjusted multivariate final model confirms that systemic JIA needed more switching due to inefficacy (2.43 [1.01-5.89], p = 0.04).</p><p><strong>Conclusion: </strong>This real-life study provides data on different switch patterns in various subtypes of JIA, confirming that patients with systemic JIA needed more switching, did more swapping strategies, and had more risk for switching due to inefficacy.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"42"},"PeriodicalIF":2.8,"publicationDate":"2025-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12023536/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144042668","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":"Unmet needs and research gaps in Still's disease across ages: proceedings from a pediatric and adult joint expert panel.","authors":"Claudia Bracaglia, Francesca Minoia, Sebastiaan J Vastert, Christoph Kessel, Lorenzo Dagna, Angelo Ravelli, Fabrizio De Benedetti","doi":"10.1186/s12969-025-01092-5","DOIUrl":"https://doi.org/10.1186/s12969-025-01092-5","url":null,"abstract":"<p><strong>Background: </strong>Still's disease (SD), including systemic juvenile idiopathic arthritis (sJIA) and adult-onset SD (AOSD), is an inflammatory condition typically characterized by daily fever, arthritis, and skin rash together with neutrophilic leukocytosis, thrombocytosis, and increased acute phase reactants. The reported differences between sJIA and AOSD appear to reflect variations along an inflammatory spectrum influenced by age, rather than differences in the underlying pathology.</p><p><strong>Methods: </strong>In February 2023, an expert meeting, including pediatric and adult rheumatologists, was held in Rome, Italy, with the aim of defining more precise and timely strategies for disease management. The following four topics were discussed: (1) early recognition and diagnosis of SD; (2) pathogenetic pathways and possible biomarkers for diagnosis and response; (3) refractory disease and risk factors, and (4) treatment of SD and its complications.</p><p><strong>Results: </strong>The development of improved diagnostic criteria and validation of biomarkers are important steps towards achieving early diagnosis, although several biomarkers remain to be universally validated and available for clinical practice. Additionally, awareness of important complications of SD, including macrophage activation syndrome and lung disease, is crucial for improving patient outcomes, alongside an improved understanding of risk factors for the development of refractory disease. While interleukin (IL)-1 and IL-6 inhibitors have improved the treatment landscape of SD, harmonizing the therapeutic approach across centers and countries, together with developing treatment strategies for refractory patients, still represents a challenge.</p><p><strong>Conclusions: </strong>Here, we summarize the results of discussions among experts, supplemented by relevant literature, and highlight unmet needs in the diagnosis and management of SD.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"40"},"PeriodicalIF":2.8,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12020313/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144057842","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}