若年性特発性関節炎

UNLABELLED: This study aims to compare intranasal dexmedetomidine with inhaled nitrous oxide for procedural pain relief and sedation during intra-articular injections in a pediatric rheumatology outpatient clinic. Patients from 1 to 18 years-of-age requiring 1-5 intra-articular injections were randomized to receive 2 µg/kg intranasal dexmedetomidine or inhaled nitrous oxide in the first visit. When the patient required subsequent treatment visit, the other sedative was administered. The primary outcome was patient-reported pain immediately after recovery from sedation. Hemodynamic and respiratory parameters, level of sedation and patient's pain assessed by the parent, rheumatologist, and nurse were secondary outcomes. Out of 119 eligible patients, 68 were included, and 43 received both medications at two separate visits. Overall pain experience was mild and did not differ between dexmedetomidine and nitrous oxide in patient-reported pain immediately after the procedure (difference, 0.2; 95% CI - 0.7 to 1.2, p = 0.65) but the patients recalled milder pain after nitrous oxide in the follow-up survey (difference, - 1.3; 95% CI - 2.3 to - 0.3; p = 0.01). Milder pain was reported during the second visit compared to the first visit regardless of the sedative used (difference, - 1.2; 95% CI - 2.1 to - 0.2; p = 0.02). In the follow-up survey, majority (69%) of patients would prefer nitrous oxide to dexmedetomidine for sedation in future. CONCLUSIONS: Both intranasal dexmedetomidine and inhaled nitrous oxide offered effective analgosedation for intra-articular injections in children. Nitrous oxide resulted in better patient experience and became their preference for future sedation. Trial registration: ClinicalTrials.gov TRN: NCT03069638. https://clinicaltrials.gov/study/NCT03069638 . The date of registration 2016-12-20. WHAT IS KNOWN: • Children suffering juvenile idiopathic arthritis often require repeated intra-articular injections. • Procedural sedation is being used during these procedures. WHAT IS NEW: • When intranasal dexmedetomidine and nitrous oxide was compared in a crossover design both agents provided effective analgosedation without difference in patient-reported pain immediately after the procedure. • Despite this, most patients favored nitrous oxide for future sedation.

Pain is a frequent symptom in people with inflammatory arthritis (IA), which has substantial impact on their quality of life. Analyses of electronic health record data indicate that UK pain care in people with IA often involves prescribing long-term opioids and gabapentinoids, despite absent trial evidence for efficacy. Patient survey data suggest that non-pharmacological pain care with supportive trial evidence is underused. A UK-specific guideline on pain management for people with IA is required to address this. This comprehensive life-course guideline is the first British Society for Rheumatology Guideline to specifically address pain in people with IA. It provides evidence-based recommendations on how pain can be best managed in people with IA. It was developed using the methods outlined in the British Society for Rheumatology's 'Creating Clinical Guidelines' protocol by a multidisciplinary Guideline Working Group, comprising healthcare professionals with expertise in paediatric and adult rheumatology and people with lived experience. By undertaking and considering the evidence from several systematic literature and umbrella reviews, 23 recommendations were developed. These address how pain should be assessed in people with IA alongside the role of the following treatments in IA pain management: DMARDs, glucocorticoids, analgesics, neuromodulators, exercise and physical activity, psychological interventions, ergonomic and orthotic interventions (excluding orthoses for foot pain), education, weight management and diet, addressing sleep problems, fatigue management, digital technologies and medical devices, complementary therapies, and support from others. An audit tool is provided to support the Guideline's implementation, and key recommendations made for future research.

OBJECTIVES: To evaluate the impact of juvenile idiopathic arthritis (JIA) on the lives of children and young adults in Australia, including healthcare interactions, treatment, physical and mental health, social/familial impact, costs and quality of life, and to determine priorities for research. STUDY DESIGN: Cross-sectional online survey conducted from February through June 2023 across Australia of individuals 0-25 years diagnosed with JIA or their parents/caregivers. Analyses included descriptive statistics: percentages and counts for discrete variables and mean and standard deviation for quantitative variables. Full text responses were coded into themes to quantify responses. RESULTS: Of 184 participants, mean age was 12 years; 67% were female. Participants averaged 25 visits annually to 5.6 health professionals. Blood tests and eye examinations averaged 4 and 3 per year, respectively. Over half (56%) had a hospitalization in the past year, of which 80% were day-stays. Medication use was high (97%), mostly csDMARDS (73.4%), NSAIDs (73.4%) and bDMARDS (53.3%) with adverse effects affecting 72%. The greatest health impact was on mental health (53%); half experienced moderate-severe pain and 30% required aids for daily living. Children missed 1 month of school/year. The greatest social impact on participants was sport (77%) and on families emotional health (59%). Mean annual government costs were 19,795 USD/participant. Quality of life measures were low with mean PedsQL score 54.5 and mean CHU9D score 0.53. Priority research areas included long-term health impact, medication use, and physical impact. CONCLUSION: The broad burden of JIA highlights the need for models of holistic management that extend beyond clinical considerations to encompass and address the lived experience and needs of individuals and families affected by JIA.

Synovial arteriovenous malformation (SAVM) of the knee is an exceedingly rare vascular anomaly, often presenting a diagnostic challenge in pediatric populations due to its nonspecific symptoms of chronic pain and recurrent swelling. We present the case of a 10-year-old boy with a 15-month history of intermittent right knee pain and difficulty bearing weight, despite normal initial radiographs. Advanced multiplanar MRI revealed an ill-defined intra-articular lesion within Hoffa's fat pad. The imaging features was not specific, and the differential diagnosis include AVM and Tenosynovial Giant Cell Tumor (TGCT). Following surgical excision, histopathological evaluation confirmed a synovial arteriovenous malformation. This case emphasizes that while SAVM mimics common conditions like juvenile idiopathic arthritis or TGCT.

BACKGROUND: Lung disease associated with systemic juvenile idiopathic arthritis (SJIA-LD) remains poorly understood. Evaluation of bronchoalveolar lavage fluid (BALF) may better reflect disease pathogenesis. The objectives of our study were to measure levels of cytokines and chemokines in BALF and their associations with clinical features and treatment in patients with SJIA-LD. METHODS: Children with SJIA-LD undergoing clinically indicated diagnostic bronchoscopy were enrolled. Comparator BALF was collected from patients with other chronic inflammatory and non-inflammatory lung diseases. BALF was assayed for IL-6, 8 and 18, S100A8/9, S100A12, sCD25, CCL11, CCL17, CCL25, MMP7, and CXCL9. RESULTS: BALF was obtained from 21 patients with SJIA-LD and 54 comparator patients with other lung diseases. Compared to all controls, children with SJIA-LD had a significant elevation of IL-18 (median (IQR) 1406 (722.3-2812) vs 37.2 (25.3-70) pg/mL, p < 0.0001), S100A8/9 (4745.3 (3003-11506) vs. 1297.5 (260-7755) ng/mL, p = 0.02), sCD25 (31.6 (19-50.3) vs. 13.6 (8.6-37.3) pg/mL, p = 0.04), MMP-7 (18,466.7 (10,462.2-33,516.1) vs. 13.645 (8.6-37.3) pg/mL, p = 0.0384), and CCL17 (0 (0-63.1; p = 0.038) vs 0 (0-0) pg/mL. BALF IL-18 was significantly higher in children with SJIA-LD compared to all control subgroups (inflammatory and non-inflammatory airway disease, autoimmune pulmonary alveolar proteinosis, and poorly controlled asthma), in patients who were actively treated with anti-cytokine biologics (N = 9), and in patients who ultimately underwent hematopoietic stem cell transfer (N = 8). Patients receiving anti-cytokine biologics also had significant elevations in several other cytokines compared to patients without such treatments, while profiles in those treated with JAKi (N = 14) were largely similar. Linear regression analysis showed an association between BALF level of IL-18 and the number of immunosuppressive medications utilized (p = 0.0167), but not with O2 requirement, dose of anakinra or prednisone, plasma IL-18, ferritin, CRP or ESR. CONCLUSIONS: Patients with SJIA-LD showed a distinct BALF cytokine profile, including significant IL-18, S100A8/9 protein, sCD25, and CCL-17 elevation. The level of IL-18 was higher in patients who required more immunosuppressive medications and were actively treated with anti-cytokine biologics. The relationship between BALF cytokine profiles and anti-cytokine biologics and JAK inhibitors is unclear and should be evaluated further.