Pediatric Rheumatology最新文献

{"title":"Joint fluid multi-omics improves diagnostic confidence during evaluation of children with presumed septic arthritis.","authors":"Yolanda Peña-López, Naureen G Tareen, Bo Zhang, Indu Raman, Carlos A Arana, Chengsong Zhu, Yang Liu, Pratibha Selvakumar, Nicolai S C van Oers, Simrat Morris, Lora V Hooper, Lawson A B Copley, Prithvi Raj","doi":"10.1186/s12969-025-01060-z","DOIUrl":"https://doi.org/10.1186/s12969-025-01060-z","url":null,"abstract":"<p><strong>Background: </strong>An accurate diagnosis of septic versus reactive or autoimmune arthritis remains clinically challenging. A multi-omics strategy comprising metagenomic and proteomic technologies were undertaken for children diagnosed with presumed septic arthritis to advance clinical diagnoses and care for affected individuals.</p><p><strong>Methods: </strong>Twelve children with suspected septic arthritis were prospectively enrolled to compare standard of care tests with a rapid multi-omics approach. The multi-omics combined bacterial 16S rRNA metagenomics, single cell transcriptomics, and proteomics on knee joint fluid specimens. The diagnostic value of the multi-omics was ascertained relative to standard of care culture and PCR-negative results.</p><p><strong>Results: </strong>Ten children with suspected primary septic arthritis and two with acute hematogenous osteomyelitis (AHO) diagnoses were assessed. Joint fluid bacterial cultures were positive for 6/12 (50%) patients, consistent with elevated inflammatory markers (IL-4, IL-6, IL-17A, TNF-a, etc.). Metagenomic bacterial sequencing results were 100% concordant with the culture results. Six patients were culture- and PCR-negative. Multiomics analyses of the 6 culture negative patients established that 2/6 culture-negative children had inflammatory arthritis with potential Juvenile idiopathic arthritis (JIA) and 1 had post-Streptococcal Reactive Arthritis. The children without any bacteremia had autoantibodies (IgGs) in the joint-fluid targeting several nuclear antigens (i.e., dsDNA, histones, Jo-1, scl-70, Ro/SS-A, SmDs, CENP-A along with non-nuclear antigens i.e. Albumin, Collagens, Myosin, Laminin, etc. Single cell transcriptomics confirmed an abundance of CD4⁺ follicular helper T (Tfh), CD8 + T cells and B cells in the autoantibody positive subjects. The combination of 16S DNA sequencing (p = 0.006), cytokine assays (p = 0.009) and autoantibody profiling (p = 0.02) were significantly distinct between those children with and without infections. This improved the diagnostic confidence for 9 of 12 (75%) children, key for treatment decisions.</p><p><strong>Conclusions: </strong>The multiomics approach rapidly identified children with bacterial or autoimmune inflammatory conditions, improving diagnostic and treatment strategies for those with presumptive septic arthritis.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"9"},"PeriodicalIF":2.8,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143029888","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":"Monocyte STAT1 phosphorylation and treatment response of JAK inhibitors in chronic nonbacterial osteomyelitis.","authors":"Motoshi Sonoda, Keishiro Kinoshita, Nobutaka Harada, Sungyeon Park, Shunichi Adachi, Yutaro Yada, Katsuhide Eguchi, Toshifumi Fujiwara, Makiko Kido-Nakahara, Noriko Kinjo, Masataka Ishimura, Shouichi Ohga","doi":"10.1186/s12969-025-01059-6","DOIUrl":"https://doi.org/10.1186/s12969-025-01059-6","url":null,"abstract":"<p><strong>Background: </strong>Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory disease of unknown cause, predominantly affecting teens and young adults. The early diagnosis and management are challenging due to the lack of reliable diagnostic markers and the occasional intractable cases despite conventional anti-inflammatory treatments. Janus kinase (JAK) inhibitors have recently shown potential utility; however, reports on their use for pediatric patients with CNO remain limited, and no established biomarkers exist to monitor disease activity. We aimed to investigate the pathophysiology of CNO and explore the rapid testing methods for accurate diagnosis and also assessing the disease activity.</p><p><strong>Methods: </strong>We assessed intracellular phosphorylation of signal transducer and activator of transcription 1 (pSTAT1) in peripheral blood monocytes or T cells following interferon-gamma (IFNγ) stimulation, using flow cytometry in 9 patients under 15 years old with CNO. The pSTAT1 expression levels were compared with those in patients with STAT1-gain of function (STAT1-GOF) mutations (n = 5), other autoinflammatory diseases (n = 7), and healthy controls. Clinical and immunological data were monitored in 4 patients with intractable CNO treated with adjunctive JAK inhibitors, focusing on scoring scales, imaging data, lymphocyte subsets, cytokine profiles, and pSTAT1 levels.</p><p><strong>Results: </strong>Monocyte pSTAT1 expression after IFNγ stimulation was elevated at diagnosis or during active CNO, similar to levels observed in STAT1-GOF cases. The pSTAT1 levels in CNO patients were significantly higher than those in other autoinflammatory diseases (p = 0.024) or controls (p < 0.001). Notably, pSTAT1 levels in CNO monocytes fluctuated with disease activity, decreasing in 5 patients during clinical remission following conventional therapies (p = 0.016). In four intractable cases, pSTAT1 levels remained high despite conventional treatments but significantly decreased after initiating JAK inhibitors (p = 0.036). This reduction correlated with improved patient pain visual analog scale (p = 0.008), CNO clinical disease activity score (p = 0.029), and better bone and joint imaging, though cytokine levels remained unchanged.</p><p><strong>Conclusions: </strong>The monocyte pSTAT1 levels after IFNγ stimulation reflect the activity of CNO, indicating the diagnostic utility as well as the monitoring effect of disease control. Adjunctive JAK inhibitors successfully controlled inflammation in treatment-resistant cases. Rapid pSTAT1 testing may help reduce osteo-articular complications, although the long-term adverse effects and resistance should be further investigated.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"6"},"PeriodicalIF":2.8,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143029881","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":"A qualitative study exploring experiences of treatment in paediatric rheumatology - children's, young people's, parents' and carers' perspectives.","authors":"Kassie Gracella Putri, Sunil Sampath, Charlotte Lucy Richardson, Alice McCloskey, Adam Pattison Rathbone","doi":"10.1186/s12969-025-01063-w","DOIUrl":"https://doi.org/10.1186/s12969-025-01063-w","url":null,"abstract":"<p><strong>Background: </strong>There is limited literature in paediatric rheumatology describing holistic lived experiences of medical treatment from perspectives of children and young people (CYP) and their parents or carers (PC). This is important as it could have implications for adherence. This study aimed to explore treatment experiences of CYP and PC in a paediatric rheumatology service.</p><p><strong>Methods: </strong>Participants were recruited at a day-case unit for intravenous infusions at a tertiary paediatric rheumatology centre. Joint qualitative semi-structured interviews with CYP and PC were used to collect data. Data were transcribed, quality checked and thematically analysed using NVivo 12.4 to identify findings.</p><p><strong>Results: </strong>Thirty-two participants (15 CYP between the ages of 6 and 16 years, 17 PC) took part in interviews lasting 41 min and 43 s, on average. Participants described experiences using infliximab, followed by tocilizumab and abatacept. Participants experienced a wave, oscillating between positive and negative trajectories. Experiences of medical treatments were described as temporary, eventually changing and leading to treatment changes or cessation. Behaviours were influenced through somatic factors (pain, function), social factors (advice from health professionals, encouragement from friends, family and teachers, practicality of using treatment in relation to school, work and finance) and cognitive factors (fear of needles, fear of specific medications, beliefs about necessity).</p><p><strong>Conclusions: </strong>Collectively, findings demonstrate experiences of medical treatment reflect the nature of many paediatric rheumatology conditions, oscillating between periods of positive and negative trajectories. Somatic, social and cognitive experiences can be positive, when treatment is considered 'successful'. Negative somatic, social or cognitive experiences led to behaviours such as treatment non-adherence. A limitation of the study is interviews were conducted jointly with CYP and PC, which may have influenced what participants were willing to say in front of one another however this does mean findings relate to both CYP and PC and so could be suitable targets for interventions to improve adherence.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"7"},"PeriodicalIF":2.8,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143029796","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":"Frequency of remission achievement in the pre-treat-to-target decade in juvenile idiopathic arthritis.","authors":"Ana Isabel Rebollo-Giménez, Angela Pistorio, Silvia Maria Orsi, Francesca Ridella, Elena Aldera, Luca Carlini, Valentina Natoli, Marco Burrone, Silvia Rosina, Roberta Naddei, Alessandro Consolaro, Esperanza Naredo, Angelo Ravelli","doi":"10.1186/s12969-025-01057-8","DOIUrl":"https://doi.org/10.1186/s12969-025-01057-8","url":null,"abstract":"<p><strong>Background: </strong>Over the past two decades there has been a remarkable advance in the management of juvenile idiopathic arthritis (JIA), which has led to considerable improvement in prognosis. In 2018, the introduction of the treat-to-target (T2T) strategy in JIA has been advocated to further ameliorate disease outcome. To provide a benchmark for comparing future outcomes in the \"T2T era\", this study investigates the percentage of JIA patients who achieved clinical inactive disease (CID) in the decade that preceded the publication of the T2T recommendations in JIA.</p><p><strong>Methods: </strong>The clinical charts of all JIA patients followed at the study center between 2007 and 2017 who were first seen within 6 months after disease onset and had a minimum of 6-month follow-up information available were reviewed retrospectively. The attainment of CID, defined by 2004 Wallace criteria, was assessed cross-sectionally at 6, 12, 24, and 60 months after first observation.</p><p><strong>Results: </strong>A total of 394 patients were included. Patients were classified into four \"functional phenotypes\": systemic arthritis (7.1%), oligoarthritis (48.2%), polyarthritis (40.4%), and other arthritis (4.3%). The overall frequency of CID was 25.1% at 6 months, 34.5% at 12 months, 44.6% at 24 months, and 49.1% at 60 months. The systemic and oligoarticular subgroups had the highest rates of CID at 6 months (32.1% and 29.5%, respectively) and at 12 months (40% and 41.1%, respectively). At the 60-month evaluation, which was available for 226 out of 394 patients (57.4%), the frequency of CID among patients still followed at study center was 42.9%, 51.7%, 46.7%, and 45.5% for the systemic, oligoarticular, polyarticular, and other arthritis phenotypes, respectively.</p><p><strong>Conclusion: </strong>A sizeable proportion of patients treated in the decade preceding the beginning of the \"T2T era\" and on continued follow-up did not achieve or maintain the state of CID over the long term. Future studies will determine whether the application of the T2T strategy increases the ability to achieve sustained disease quiescence in patients who respond suboptimally to the conventional therapeutic regimens.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"8"},"PeriodicalIF":2.8,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143029804","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":"Experiences of sexual and reproductive health screening and counseling in the clinical setting among adolescents and young adults with rheumatic disease.","authors":"Brittany Huynh, Mary A Ott, Stacey E Tarvin","doi":"10.1186/s12969-025-01056-9","DOIUrl":"10.1186/s12969-025-01056-9","url":null,"abstract":"<p><strong>Background: </strong>Our objective was to describe differences among adolescents and young adults (AYAs) with rheumatic disease using teratogens compared to non-users in receipt of sexual and reproductive health (SRH) counseling, teratogenicity knowledge, perceived importance of SRH topics, and preferences around counseling.</p><p><strong>Methods: </strong>AYAs ages 14-23 years and assigned female at birth were recruited from pediatric rheumatology clinics at a Midwest tertiary care program. Participants completed a one-time online survey assessing SRH.</p><p><strong>Results: </strong>One-hundred eight participants completed the survey, representing a range of rheumatic diseases. 24% reported ever having sex. 36% used a teratogen. Rates of screening and counseling regarding SRH topics were low. Notably, pregnancy prevention and emergency contraception (EC) counseling by rheumatologists were uncommon and not associated with teratogen use or sexual activity. Among AYAs on teratogens, only half reported screening for sexual activity or counseling on teratogenicity or pregnancy prevention. Gaps in pregnancy prevention and EC counseling remained even when accounting for counseling by other providers. Knowledge of medication teratogenicity was also low. AYAs reported SRH topics of high importance, and many reported recent concerns. They preferred to receive information from their rheumatologist, and most agreed it is important to talk to their rheumatologist regarding these topics.</p><p><strong>Conclusions: </strong>AYAs with rheumatic disease report low levels of SRH screening and counseling by their rheumatologist yet report these topics are important and want to discuss them. Gaps in teratogenicity knowledge were identified. This study identifies a need for improved communication with AYAs regarding their SRH.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"5"},"PeriodicalIF":2.8,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11749332/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143016469","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":"Reducing the risk of visual disability for children with juvenile idiopathic arthritis uveitis through disease surveillance: past and future challenges.","authors":"Ameenat Lola Solebo, Ivan Foeldvari","doi":"10.1186/s12969-024-01037-4","DOIUrl":"10.1186/s12969-024-01037-4","url":null,"abstract":"<p><p>Childhood blindness significantly impacts development, education, employment, and mental health, creating burden for families and society. Between 8% and 30% of children with Juvenile Idiopathic Arthritis (JIA) develop a potentially blinding chronic inflammatory eye disease, uveitis (JIAU). Alongside the use of disease-modifying agents and anti-TNF immunomodulators, JIAU surveillance has helped to reduce the risk of JIAU related blindness.Inconsistent guidance on JIAU surveillance has previously been a hindrance to care delivery and access for professional and families. The Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC) has brought some much-needed standardisation to JIA surveillance, developing a consensus-based screening proposal which simplifies the protocol, supporting implementation amongst non-specialists, and ensuring that children at risk receive the timely eye examination necessary to avoid life-changing visual disability. In this commentary on the MIWGUC surveillance proposal, we also address the implementation of such surveillance. A global shortage of ophthalmologists threatens the sustainability of these surveillance programs. Innovative approaches could be imaging-based detection. The accessibility of Optical Coherence Tomography (OCT) imaging may make OCT a feasible future option for community-based surveillance, reducing the burden on ophthalmologists, and on patients and their families.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"4"},"PeriodicalIF":2.8,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11744860/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143016471","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":"Increasing and maintaining rates of standardized depression screening in youth with childhood-onset systemic lupus erythematosus in a pediatric rheumatology clinic.","authors":"Emily Datyner, Jodi Dingle, Victoria Newsome, Lisa H Buckley, Natasha Belsky, Seungweon Park, Manda Mitchell, Brooke Fine, Barron Patterson, T Brent Graham, Alaina Davis","doi":"10.1186/s12969-024-01038-3","DOIUrl":"10.1186/s12969-024-01038-3","url":null,"abstract":"<p><strong>Background: </strong>Depression adversely affects health outcomes in patients with childhood-onset systemic lupus erythematous (cSLE). By identifying patients with depressive symptoms, we can intervene early with referrals to mental health resources and improve outcomes. The aim of our quality improvement project was to increase and maintain rates of standardized depression screening for youth with cSLE seen within our pediatric rheumatology clinic.</p><p><strong>Methods: </strong>Patients with cSLE 12 years of age or older seen for routine follow-up at our pediatric rheumatology clinic from September 16, 2019, through December 30, 2022, were offered the Patient Health Questionnaire-9 modified for adolescents (PHQ-A) to screen for depressive symptoms. A multidisciplinary team developed a key driver diagram to plan potential interventions to improve rates of screening. Plan‒Do‒Study‒Act (PDSA) cycles were used to prepare, implement, and evaluate interventions. Notable interventions focused on accurately identifying eligible patients, facilitating bidirectional communication between staff, and integrating and automating screening within the electronic health record (EHR). Statistical process control (SPC) methods were used for data analysis.</p><p><strong>Results: </strong>The percentage of eligible patient encounters where depression screening was completed increased from 0 to 81% and was maintained for more than 6 months. This represents special cause variation, as evidenced by data shifts on our statistical process control chart. Among the 592 patients who completed depression screens, 114 (17%) were positive for moderate to severe symptoms, and 59 (9%) were positive for suicidal ideation (SI).</p><p><strong>Conclusions: </strong>A high rate of standardized depression screening for youth with cSLE was achieved and maintained via integration and automation within our EHR. Establishing a highly reliable screening system is a critical first step in improving mental health care for this vulnerable population of youth.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"3"},"PeriodicalIF":2.8,"publicationDate":"2025-01-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11699778/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142928784","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 associated with treatment response in chronic nonbacterial osteomyelitis at a single center: a retrospective cohort study.","authors":"Katherine D Nowicki, Nathan D Rogers, Carson L Keeter, Nathan J Donaldson, Jennifer B Soep, Yongdong Zhao","doi":"10.1186/s12969-024-01051-6","DOIUrl":"10.1186/s12969-024-01051-6","url":null,"abstract":"<p><strong>Background: </strong>NSAIDs are commonly used as first line therapy in chronic nonbacterial osteomyelitis (CNO) but are not effective for all patients. The objective of this study was to identify clinical variables associated with NSAID monotherapy response versus requiring second-line medication in a single-center cohort of patients with CNO.</p><p><strong>Methods: </strong>The charts of children with CNO who attended a CNO clinic at a quaternary care center between 1/1/05 and 7/31/21 were retrospectively reviewed. Patients were divided into 3 groups: NSAID-short (NSAID monotherapy for 3 to < 7 months), NSAID-long (NSAID monotherapy for ≥ 7 months), or second-line treatment. Patients were also categorized by which bodily regions were affected by CNO. Multiple linear and logistic regression models were constructed to predict total NSAID monotherapy days and the odds of needing second-line treatment, respectively. These models were optimized using variable combinations that minimized multicollinearity and maximized predictive power, as indicated by minimized AIC values.</p><p><strong>Results: </strong>One-hundred-sixty-four patients fulfilled inclusion criteria. Thirty-two patients were in the NSAID-short group, 62 in the NSAID-long group, and 70 in the second-line treatment group. Comparing the two NSAID groups showed that patients with unifocal disease at diagnosis required 47% fewer days of NSAIDs than those with multifocal disease. Results from logistic regression indicated that for each additional region affected, the odds of needing second line treatment increased by 1.94 times (p = 0.01) and that patients with symmetric bone lesions were 6.86 times more likely to require second-line treatment (p < 0.001).</p><p><strong>Conclusions: </strong>Patients with unifocal CNO involvement at diagnosis were more likely to require shorter NSAID treatment. Patients with more regions affected and those with symmetric bone lesions were more likely to require second-line treatment.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"2"},"PeriodicalIF":2.8,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11697481/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142928782","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":"Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a case-based review emphasizing management.","authors":"Batuhan Küçükali, Merve Yazol, Çisem Yıldız, Büşra Acun, Nuran Belder, Nihal Karaçayır, Merve Kutlar, Pelin Esmeray Şenol, Zühre Kaya, Deniz Gezgin Yıldırım, Sevcan A Bakkaloğlu","doi":"10.1186/s12969-024-01054-3","DOIUrl":"10.1186/s12969-024-01054-3","url":null,"abstract":"<p><strong>Background: </strong>Pediatric patients with Eosinophilic Granulomatosis with Polyangiitis (EGPA) are at an increased risk of arterial and venous thromboembolism (AVTE). Although the exact mechanisms underlying AVTE remain unclear, eosinophils play a pivotal role in AVTE.</p><p><strong>Main body: </strong>Current guidelines lack evidence-based recommendations, particularly concerning anticoagulant and antiplatelet treatments for this condition. Herein, we document a pediatric EGPA patient with deep venous thrombosis presenting with massive pulmonary thromboembolism during a relapse, treated with immunosuppressive and anticoagulant therapy to raise awareness among clinicians. Additionally, we performed a literature review to highlight various aspects of pediatric AVTE. Moreover, we evaluated the management strategies employed for the patients identified in the literature review and summarized the current practice guidelines regarding pediatric EGPA patients with AVTE to provide recommendations to clinicians on the management of this challenging complication.</p><p><strong>Conclusions: </strong>Most AVTE events occur during periods of high disease activity. Notably, EGPA patients with VTE often present with thrombocytopenia due to consumption, a finding not typically expected during disease exacerbation. Venous thrombosis generally requires both anticoagulation and immunosuppressive treatment. Although our review indicates a favorable prognosis for AVTE, the small number of reported cases prevents us from drawing definitive conclusions. Future studies should explore the efficacy of mepolizumab and other eosinophil-targeted therapies for AVTE, in addition to investigating the roles of anticoagulation and antiplatelet treatments.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"1"},"PeriodicalIF":2.8,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11697824/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142928786","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":"Approaches and outcomes of adalimumab discontinuation in patients with well-controlled inflammatory arthritis: a systematic search and review.","authors":"Erin Balay-Dustrude, Jessica Fennell, Kevin Baszis, Y Ingrid Goh, Daniel B Horton, Tzielan Lee, Chloe Rotman, Anna Sutton, Marinka Twilt, Olha Halyabar","doi":"10.1186/s12969-024-01046-3","DOIUrl":"10.1186/s12969-024-01046-3","url":null,"abstract":"<p><strong>Objective: </strong>This systematic search and review aimed to evaluate the available literature on discontinuation of adalimumab and other tumor necrosis factor inhibitors (TNFi) for patients with well-controlled chronic inflammatory arthritides.</p><p><strong>Methods: </strong>We conducted a publication search on adalimumab discontinuation from 2000-2023 using PubMed, CINAHL, EMBASE, and Cochrane Library. Included studies evaluated adalimumab discontinuation approaches, tapering schemes, and outcomes including successful discontinuation and recapture after flare, in patients with well-controlled disease. Studies included evaluated rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, and juvenile idiopathic arthritis (JIA).</p><p><strong>Results: </strong>Forty-nine studies were included. Studies evaluating adalimumab alone were limited, and many reported TNFi outcomes as a single entity. Studies on rheumatoid arthritis (RA) (32, 8 RCTs) reported flare rates from 33-87%. Flares with medication tapering were slightly lower than with abrupt stop, and successful recapture was generally high (80-100%). Studies on spondyloarthropathy (12, 4 RCTs), focused on tapering, noting lower flare rates in tapering rather than abruptly stopping, and high recapture rates (~ 90%). Studies on JIA (5) were observational and demonstrated modestly lower flare rates with tapering (17-63%) versus abrupt stopping (28-82%). There was notable variability in study design, follow-up duration, specificity for TNFi results, and controlled pediatric studies.</p><p><strong>Conclusion: </strong>The literature evaluating adalimumab and other TNFi discontinuation, flare rates, and recapture success within the inflammatory arthritis population demonstrated less flare when medications were tapered, over abrupt stop in the RA, spondyloarthropathy, and JIA populations. When medications were restarted after flare, recapture of well-controlled disease was generally high in RA and spondyloarthropathy, and generally favorable in JIA.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"112"},"PeriodicalIF":2.8,"publicationDate":"2024-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11684048/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142904010","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}