Paediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS) is a novel condition that was first reported in April, 2020. We aimed to develop a national consensus management pathway for the UK to provide guidance for clinicians caring for children with PIMS-TS. A three-phase online Delphi process and virtual consensus meeting sought consensus over the investigation, management, and research priorities from multidisciplinary clinicians caring for children with PIMS-TS. We used 140 consensus statements to derive a consensus management pathway that describes the initial investigation of children with suspected PIMS-TS, including blood markers to help determine the severity of disease, an echocardiogram, and a viral and septic screen to exclude other infectious causes of illness. The importance of a multidisciplinary team in decision making for children with PIMS-TS is highlighted throughout the guidance, along with the recommended treatment options, including supportive care, intravenous immunoglobulin, methylprednisolone, and biological therapies. These include IL-1 antagonists (eg, anakinra), IL-6 receptor blockers (eg, tocilizumab), and anti-TNF agents (eg, infliximab) for children with Kawasaki disease-like phenotype and non-specific presentations. Use of a rapid online Delphi process has made it possible to generate a national consensus pathway in a timely and cost-efficient manner in the middle of a global pandemic. The consensus statements represent the views of UK clinicians and are applicable to children in the UK suspected of having PIMS-TS. Future evidence will inform updates to this guidance, which in the interim provides a solid framework to support clinicians caring for children with PIMS-TS. This process has directly informed new PIMS-TS specific treatment groups as part of the adaptive UK RECOVERY trial protocol, which is the first formal randomised controlled trial of therapies for PIMS-TS globally.
The PVAS validity in children with systemic vasculitis was demonstrated. Like the BVAS, we anticipate that the PVAS will provide a robust tool to objectively define disease activity for clinical trials and future research.
Eighteen Caucasian (white, Middle East and Asian) children diagnosed by paediatric rheumatologists in the UK and France as having systemic juvenile idiopathic arthritis (sJIA) were enrolled in this open label, single dose trial. All patients had evidence of continued symptoms and disease activity for at least three months while receiving >0.2 mg/kg/day of prednisolone, or its equivalent, prior to recruitment. Twelve patients also received methotrexate (≤20 mg/m 2 /week). The patients were divided into three groups receiving 2, 4 or 8 mg/kg of MRA (tocilizumab) by intravenous infusion. No evidence of doselimiting toxicity was observed and there were no dose-limiting safety issues. MRA appeared to be dramatically effective, with clinical and laboratory responses observed by 48 h post infusion, and these improvements continued well after serum MRA was undetectable. Eleven patients achieved the JIA definition of improvement (at least 3 of 6 core set criteria with a 30% improvement and no more than one worsened by 30%) and eight achieved ≥50% improvement. There were no observable differences with age. Clinical improvement in these children was observed for up to eight weeks, supporting the hypothesis that IL-6 is a key cytokine in the upregulation of genes crucial in the inflammation processes of sJIA, and the possibility of sequestration of MRA in the extra-vascular compartment needs to be considered.
Aims: To examine the contribution of demographic, social, clinical and psychological factors to emotional distress in patients with painful diabetic neuropathy (DN). Methods: In total, 142 patients with confirmed painful DN underwent detailed clinical and self-assessment measures (Neuropathic Pain Scale, Hospital Anxiety and Depression Scale, Pain Acceptance Questionnaire and Pain Catastrophizing Scale). Results:The prevalence of emotional distress was 51.4% in this cohort. Age, sex, marital status, employment history, pain intensity, duration of diabetes and the presence of diabetic and non-diabetic complications were significantly correlated to anxiety and depressive symptom scores. Multiple regression analysis confirmed that the presence of catastrophic thinking was an independent contributor to greater symptoms of anxiety and depression. Being young, single and unemployed significantly contributed to greater anxiety symptoms. Pain-related restriction of quality of life was associated with greater depression symptom scores. Conclusions: This study found a high prevalence of emotional distress in patients with painful DN. It highlights that the differing independent contributors to anxiety and depressive symptoms are based on an individual's circumstances and experience. We conclude by highlighting the importance of adopting a holistic approach to pain management, incorporating interventions to increase psychological flexibility alongside conventional pharmacological treatments to improve emotional distress in painful DN.
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