Pediatric Rheumatology最新文献

{"title":"Serositis as an indicator of poor prognosis in pediatric systemic lupus erythematosus.","authors":"Wei-Chen Kao, Ya-Chiao Hu, Jyh-Hong Lee, Li-Chieh Wang, Yu-Tsan Lin, Yao-Hsu Yang, Bor-Luen Chiang, Hsin-Hui Yu","doi":"10.1186/s12969-025-01084-5","DOIUrl":"https://doi.org/10.1186/s12969-025-01084-5","url":null,"abstract":"<p><strong>Background: </strong>Systemic lupus erythematosus (SLE) is a multi-systemic autoimmune disease that causes inflammation of the serosa (serositis). This retrospective study aimed to evaluate the clinical characteristics of serositis in childhood-onset SLE (cSLE) and analyze its association with long-term outcomes.</p><p><strong>Methods: </strong>We retrospectively reviewed the medical records of patients with cSLE diagnosed at a medical center in Taiwan, analyzing data collected from January 2002 to December 2022. We analyzed the clinical features of patients with serositis as pleuritis and/or pericarditis with at least a small effusion (> 0.5 cm in depth) on sonography or chest radiography. Cox proportional hazards regression was used to calculate the hazard ratios (HR) and 95% confidence intervals (CI) for the association between serositis and all-cause mortality.</p><p><strong>Results: </strong>185 patients with cSLE were enrolled, of whom 38 (20.54%) had serositis. Patients with serositis had a younger age at SLE diagnosis, a higher SLE Disease Activity Index 2000 score at serositis diagnosis, and an increased prevalence of lupus nephritis, central nervous system manifestations, end-stage renal disease (ESRD), a higher Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) damage index score, and a higher mortality than that of patients without serositis. Multivariate Cox regression analysis showed that both serositis (hazard ratio [HR]: 5.585, confidence interval [CI]: 1.853-17.80) and ESRD (HR: 13.956; CI: 3.822-50.964) were associated with mortality risk. Kaplan-Meier survival curve analysis revealed that patients with both serositis and ESRD had the poorest 20-year survival rate. Patients with late-onset serositis (occurring 1 year after SLE diagnosis) had higher mortality rates than those with early-onset serositis.</p><p><strong>Conclusion: </strong>Children with lupus serositis had higher disease activity, a higher prevalence of comorbidities, and mortality. Patients with both serositis, especially late-onset serositis, and ESRD had an increased risk of poor long-term survival.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"36"},"PeriodicalIF":2.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143765812","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}

{"title":"Case report: novel NFKB2 variant associated with pediatric eosinophilic granulomatosis with polyangiitis (EGPA) in the COVID-19 pandemic.","authors":"Li Lin, Xin Peng, Lina Chen, Liqun Dong, Lin Zhong","doi":"10.1186/s12969-025-01086-3","DOIUrl":"https://doi.org/10.1186/s12969-025-01086-3","url":null,"abstract":"<p><strong>Background: </strong>Childhood-onset eosinophilic granulomatosis with polyangiitis (cEGPA) is a rare type of systemic autoimmune disorder. Variants in the NFKB2 gene can manifest as common variable immunodeficiency or combined immunodeficiency, often accompanied by autoimmunity and ectodermal dysplasia. Here, we report a case of a Chinese patient who carries NFKB2 variants that coexist with cEGPA, a novel combination which, to our knowledge, has not been previously published.</p><p><strong>Case presentation: </strong>We reported a 9-year and 10-month-old girl who presented with cough, wheezing, dyspnea, hypereosinophilia, and vasculitis. Notably, she had significant bilateral pulmonary interstitial lesions. We performed metagenomic next-generation sequencing (mNGS), bronchoscopy and immunological analysis. She was considered to have refractory cEGPA after six months of corticosteroid and immunosuppressive treatment. Tapering off corticosteroids posed a challenge, and multiple immunosuppressive agents were ineffective. Our patient suffered from recurrent fever, wheezing, dyspnea and perianal abscess, along with life-threatening infections, including pneumocystis jirovecii pneumonia (PJP) and severe coronavirus disease 2019 (COVID-19) pneumonia during the pandemic. Her cytokines and inflammatory markers showed a profound collapse. She developed significant hypoxemia, which necessitated mechanical ventilation. Primary immunodeficiency gene panel testing revealed a novel de novo variant in NFKB2 (c.2578 + 2 dup) that was classified as pathogenic. Despite treatment with antibacterial, antiviral, and antifungal agents, biologics, and plasma exchange, she ultimately succumbed to respiratory failure.</p><p><strong>Conclusions: </strong>This case report establishes a novel link between NFKB2 variants and EGPA, particularly in the context of the COVID-19 pandemic. This study expands the spectrum of NFKB2 variants and vividly illustrates the complex interrelationships among autoimmunity, infection, and immunodeficiency.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"35"},"PeriodicalIF":2.8,"publicationDate":"2025-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143756019","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}

{"title":"Epidemiology and comorbidity of juvenile idiopathic arthritis in Poland- a nationwide study.","authors":"Zbigniew Żuber, Krzysztof Podwójcic, Mateusz Szeląg, Magdalena Krajewska-Włodarczyk, Krzysztof Batko, Michał Orleański, Jakub Sowiński, Maria Świderek, Agata Śmiglewska, Michał Maluchnik, Marek Brzosko, Brygida Kwiatkowska, Marcin Stajszczyk, Bogdan Batko","doi":"10.1186/s12969-025-01065-8","DOIUrl":"https://doi.org/10.1186/s12969-025-01065-8","url":null,"abstract":"<p><strong>Background: </strong>Diagnostic pathways for patients with juvenile idiopathic arthritis (JIA) have gradually improved over time. Provider practice has also shifted towards goal-oriented treatment with disease-modifying drugs (DMARDs) that together may have changed the epidemiologic landscape of JIA.</p><p><strong>Methods: </strong>Public healthcare utilization records from the National Health Fund (NHF) were screened between 2010 and 2022. For individuals aged < 16 years, we utilized a narrow JIA case definition combining repeat ICD-10 encoding with DMARDs prescription based on ATC codes.</p><p><strong>Results: </strong>In 2022, we identified 1,625 incident and 29,758 prevalent JIA cases (< 16 years), which corresponds to incidence (IRs) and prevalence rates of 4.30 and 78.80 per 100,000 persons of the general population. For the pediatric population, annual IRs for JIA (< 16 years) ranged between 24.0 (95% CI 22.8, 25.2) and 38.7 (95% CI 37.2-40.3) per 100,000. Greater susceptibility among females was also consistently observed with the annual IR ratio ranging between 1.16 and 1.53. The most common concurrent disorders based on medical care services were allergic rhinitis (N = 5,200, 17.5%), bronchial asthma (N = 3,661, 12.3%) and chronic tonsillitis/pharyngitis (N = 3641, 12.2%). Analysis of 214,285 outpatient care visits revealed a median (IQR, range) annual healthcare cost of 37.8€ (35.8-47.4€, 30.3-86.1€) per JIA patient.</p><p><strong>Conclusions: </strong>This comprehensive, nationwide study provides a contemporary estimate of JIA burden in Poland. Our findings indicate that both the occurrence of new cases and overall burden of JIA in the past ten years align with the lower end of projected figures for our geographical area, especially when compared with Scandinavian nations.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"33"},"PeriodicalIF":2.8,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11951515/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143744579","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":"Perspectives and experiences of parents of children with juvenile dermatomyositis: a semi-structured interview study.","authors":"Amy Helen Kelly, Ayano Kelly, Davinder Singh-Grewal, Jeffrey Chaitow, Allison Jaure","doi":"10.1186/s12969-025-01079-2","DOIUrl":"https://doi.org/10.1186/s12969-025-01079-2","url":null,"abstract":"<p><strong>Background: </strong>Juvenile Dermatomyositis (JDM) is a rare, childhood inflammatory disease and its management can be challenging and confronting for both clinicians and caregivers. Little is known about the perspectives of parental caregivers of children with JDM. This study aimed to describe the experiences of parents of children with JDM to inform person-centred care.</p><p><strong>Methods: </strong>Semi-structured interviews (face-to-face, telephone) were conducted with parents of children with JDM from three centres in Australia. Transcripts were analysed thematically.</p><p><strong>Results: </strong>Nineteen parents (15 mothers) of 17 children aged 8 to 21 with JDM participated. Six themes were identified. Rapid crescendo of fear and desperation (alarming deterioration, sudden realisation of seriousness, desperate for a diagnosis ), lost and unsupported in the health system (at the mercy of the medical team, frustrated at the lack of services, neglected priorities, protracted and painful search for answers), disrupting family routines (sibling neglect and loss, overloaded with a medicalised schedule, always on standby, burdened by financial strains), grieving what has been lost (missing the sunlight, struggling with the loss of physical function, disrupted schooling, changes in their child from steroid side effects), managing an uncertain future (bound to chronicity, fearing relapse, insecurity with transition to adult care), gaining confidence and motivation (strengthening partnerships with clinicians, growing maturity and independence, gaining hope from shared experiences).</p><p><strong>Conclusions: </strong>The diagnosis of JDM is often delayed and caregivers of children with JDM report distress, disruption and uncertainty throughout their treatment journey with their child. Addressing these fears and establishing support mechanisms that help parents navigate their way through the medical system and support changing family dynamics are vital to optimise health outcomes for children diagnosed with JDM.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"34"},"PeriodicalIF":2.8,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11951517/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143744580","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":"Monozygotic twins discordant for juvenile dermatomyositis: clinical, serological and gene expression studies.","authors":"Lauren M Pachman, Amer Khojah, Gabrielle Morgan, Wilfredo Marin, Judith James, Sabah Kadri, Kai Lee Yap","doi":"10.1186/s12969-025-01082-7","DOIUrl":"10.1186/s12969-025-01082-7","url":null,"abstract":"<p><strong>Background: </strong>Juvenile Dermatomyositis (JDM) is a rare pediatric autoimmune disease involving a combination of environmental and genetic susceptibility factors. Monozygotic twins provide a unique opportunity to examine disease-specific gene expression as they share the same DNA. The goal of this study is to characterize gene expression differences between monozygotic twins discordant for JDM.</p><p><strong>Methods: </strong>Five pairs of monozygotic twins were included. Each twin set was discordant for JDM. Detailed clinical and laboratory assessments were performed at enrollment. Nailfold capillary end row loops (ERL) count was obtained for all study subjects. Serum levels of cytokines and chemokines were measured using the Meso Scale Discovery^® technique. Three pairs of twins had their peripheral blood mononuclear cells (PBMCs) tested by RNASeq.</p><p><strong>Results: </strong>The JDM twin had significantly lower nailfold capillary ERL than the healthy control, and two non-JDM twins also had decreased ERL In addition, serum endoglin was significantly lower in both JDM and non-JDM twins than in the healthy control. RNASeq identified four genes differentially expressed between the JDM and non-JDM twins: DCD, KRT14, COL1A1, and COL3A1.</p><p><strong>Conclusions: </strong>JDM twins (and two of the non-JDM twins) had significantly lower nailfold capillary ERL and decreased serum endoglin levels compared to healthy controls. Further studies are needed to explore the role of the differentially expressed genes (DCD, KRT14, COL1A1, and COL3A1) in the pathophysiology of JDM.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"32"},"PeriodicalIF":2.8,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11938629/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143722619","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":"Red blood cell distribution width predicts coronary artery lesions in Kawasaki disease: insights from a Japanese cohort.","authors":"Yamato Hanawa, Wataru Murasaki, Hiroyuki Namba, Kimihiko Oishi","doi":"10.1186/s12969-025-01083-6","DOIUrl":"10.1186/s12969-025-01083-6","url":null,"abstract":"<p><strong>Background: </strong>Kawasaki disease (KD) is an acute vasculitis that causes coronary artery lesions. This study aimed to identify risk factors for the early prediction of coronary artery disease (CAD) in KD.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of 175 Japanese children diagnosed with KD between January 2019 and March 2024. Univariate and multivariate logistic regression analyses were performed to identify predictors of CAD, and the diagnostic performance of various indicators was assessed using receiver operating characteristic (ROC) curves. The correlations between red blood cell distribution width (RDW) and iron-related anemia biomarkers were also evaluated.</p><p><strong>Results: </strong>Of these, 77 with CAD were classified into the CAD group, while 98 without CAD were categorized as the non-CAD group. Patients in the CAD group were younger and had lower levels of hemoglobin (Hb), total protein, albumin, uric acid, and urea nitrogen, but a higher RDW coefficient of variation (RDW-CV) than the non-CAD group. Logistic regression analysis identified RDW-CV as an independent predictor of CAD. ROC curve analysis demonstrated moderate predictive performance for RDW-CV, with an area under the curve of 0.636 (sensitivity, 55.8%; specificity, 70.4%). Significant correlations were observed between RDW-CV and iron-related anemia biomarkers in the CAD group, but not in the non-CAD group.</p><p><strong>Conclusions: </strong>Iron dysregulation may be associated with CAD, and RDW-CV may aid in identifying patients who may develop CAD in KD. Our findings were consistent with previous studies in other Asian populations, supporting the utility of RDW-CV as a predictor of CAD in KD in populations with various ethnic backgrounds.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"31"},"PeriodicalIF":2.8,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11934559/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143712176","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":"Clinical insights into heterogeneity of rheumatoid factor negative polyarticular juvenile idiopathic arthritis across the world.","authors":"Roberta Naddei, Marco Burrone, Francesca Ridella, Yosef Uziel, Maria Trachana, Pavla Dolezalova, Ingrida Rumba-Rozenfelde, Nicolino Ruperto, Angelo Ravelli, Alessandro Consolaro","doi":"10.1186/s12969-025-01072-9","DOIUrl":"10.1186/s12969-025-01072-9","url":null,"abstract":"<p><strong>Background: </strong>To our knowledge, limited information is available about the differences in the characteristics of rheumatoid factor (RF)-negative polyarticular juvenile idiopathic arthritis (JIA) throughout the world. This study was aimed to compare the demographic and clinical features of patients with RF-negative polyarthritis across the world.</p><p><strong>Methods: </strong>Patients were part of a multinational sample included in a study aimed to investigate the prevalence of disease categories, treatment regimens, and disease status in patients from different geographical areas (EPOCA Study). All patients underwent a retrospective assessment, based on the review of clinical chart, and a cross-sectional evaluation, which included assessment of physician- and parent-reported outcomes and collection of ongoing medications.</p><p><strong>Results: </strong>Among the 9081 patients enrolled in the EPOCA study, 2141 patients (23.6%) with RF-negative polyarthritis were included in the present analysis. The prevalence of RF-negative polyarthritis was highest in North America and lowest in Southeast Asia (12.7%). The age at disease onset was lower in Northern and Southern Europe, where the highest prevalence of uveitis was found. Uveitis was rare in Southeast Asia, Africa & Middle East and Latin America. Patients from Eastern Europe, Latin America and Africa and Middle East presented with the highest prevalence of active joints at the visit. The combination of early onset, ANA positivity, and uveitis was observed mainly in Southern Europe (39%).</p><p><strong>Conclusions: </strong>Our results confirm the wide heterogeneity of the clinical presentation and outcome of children with RF-negative polyarticular JIA throughout the world. In particular, relevant differences in the onset age were observed across geographic areas. The group of children with early onset polyarthritis, ANA positivity, and risk of uveitis is remarkably frequent in Southern Europe.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"30"},"PeriodicalIF":2.8,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11921679/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143665367","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":"Abstracts of the 35th Meeting of the German Society for Pediatric and Adolescents Rheumatology (Gesellschaft für Kinder- und Jugendrheumatology (GKJR).","authors":"","doi":"10.1186/s12969-025-01055-w","DOIUrl":"10.1186/s12969-025-01055-w","url":null,"abstract":"","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 Suppl 1","pages":"28"},"PeriodicalIF":2.8,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11912620/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143651911","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":"T-ing up the storm: pathogenic cycling lymphocytes in the biology of macrophage activation syndrome.","authors":"Michael T Lam, Connie L Jiang, Pui Y Lee","doi":"10.1186/s12969-025-01081-8","DOIUrl":"10.1186/s12969-025-01081-8","url":null,"abstract":"<p><strong>Background: </strong>Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are potentially fatal cytokine storm syndromes with clinical features including fever, pancytopenia, hepatosplenomegaly, coagulopathy, and progressive multiorgan system dysfunction. Mechanistically, HLH / MAS are driven by persistent activation of lymphoid and myeloid cells, but our understanding of the pathogenic cell populations remains incomplete.</p><p><strong>Main body: </strong>In this Perspectives article, we provide an overview of the biology of HLH / MAS and the critical role of interferon-g in disease pathogenesis. We discuss the recent discovery of cycling lymphocytes in HLH / MAS marked by expression of CD38 and HLA-DR, which are primary producers of IFN-γ. The expansion of cycling lymphocytes correlates with disease activity and helps to distinguish HLH / MAS from clinical mimics. We demonstrate an approach to quantify CD38⁺HLA-DR⁺ cycling lymphocytes and evaluate their utility as a diagnostic biomarker for HLH / MAS. Lastly, we discuss the treatment of MAS, including potential therapeutic options to target these pathogenic lymphocytes.</p><p><strong>Conclusion: </strong>Understanding of biology of cycling lymphocytes in HLH / MAS will facilitate the development of novel therapeutic approaches to overcome these fatal hyperinflammatory disorders.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"29"},"PeriodicalIF":2.8,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11912701/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143651909","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":"African guidelines for diagnosis and management of polyarticular juvenile idiopathic arthritis: PAFLAR initiative.","authors":"Mohammed Hassan Abu-Zaid, Angela Nyangore Migowa, Hanna Lishan Kassa, Wassila Messadi, Yassmine Taha, Yaninga Halwani Fuseini, Madeleine Ngandeu, Yasser El Miedany, Michael Hofer, Wafa Hamdi, Temesgen Teferi Libe, Ali Sobh, Waleed Hassan, Yasmine Makhlouf, Ayodele Faleye, Soad Hashed, Samah Ismail Nasef, Chafia Dahou Makhloufi, Elisa Palalane, Hanene Lassoued Ferjani, Ahmed Seri, Doaa Mosad Mosa, Ourida Gacem, Francis Fredrick Furia, Samy Slimani, Christiaan Scott, Djohra Hadef","doi":"10.1186/s12969-025-01076-5","DOIUrl":"10.1186/s12969-025-01076-5","url":null,"abstract":"<p><strong>Background: </strong>Juvenile idiopathic arthritis (JIA) is the most common rheumatologic disease of childhood. The Existing guidelines for polyarticular JIA are typically based on data from non-African populations and may not fully address the unique challenges faced in African settings. We aimed to produce updated African guidelines for the diagnosis and treatment of children and adolescents with polyarticular juvenile idiopathic arthritis (poly-JIA).</p><p><strong>Methods: </strong>This study was conducted with the aim of reaching a consensus among African experts on the diagnosis and treatment of poly-JIA using the Delphi technique. The first scientific committee identified a total of 15 key clinical questions according to the PICO (Patient/Population, Intervention, Comparison, Outcome) approach. A systematic review of the evidence-based literature was conducted for this work. The core steering group identified researchers and clinicians with expertise in pediatric rheumatology. A Delphi process was used to reach consensus.</p><p><strong>Results: </strong>An online questionnaire was sent to the expert panel that participated in the survey (100% response rate). A total of 15 recommendation points were identified, divided into two parts: five recommendations for diagnosis and ten recommendations for management. The percentage of those who agreed with the recommendations (fourth and fifth place) ranged from 80 to 100%. All 15 clinical recommendation statements that the scientific committee had identified had been agreed upon in wording (i.e., 75% of respondents agreed or strongly agreed).</p><p><strong>Conclusions: </strong>We successfully developed guidelines for children with polyarticular JIA, taking into consideration the African specific nature of limited resources and low income, also on the same time incorporating newly released data and using a treat to target approach.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"27"},"PeriodicalIF":2.8,"publicationDate":"2025-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11909873/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143634965","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}