The Great North Children's Hospital (GNCH) is a tertiary referral centre in Newcastle upon Tyne, England. The hospital is managed by the Newcastle upon Tyne Hospitals NHS Foundation Trust and is a teaching hospital for the University of Newcastle upon Tyne. It is one of only 14 such children's hospitals in the United Kingdom.
OBJECTIVE:To assess the 2022 American College of Rheumatology (ACR)/EULAR classification criteria for antineutrophil cytoplasmic antibody-associated vasculitis (AAV) in children with chronic small-to-medium vessel vasculitis. METHODS:A cohort of 574 patients, identified by physician's diagnosis (MD-diagnosis) in A Registry of Childhood Vasculitis, was classified by computation of registry data as having granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or eosinophilic GPA after applying (1) ACR/EULAR AAV criteria and (2) pediatric-adapted European Medicines Agency (Ped-EMA) classification algorithm (incorporating Ankara GPA criteria). Venn diagrams compared the resulting GPA and MPA cohorts with MD-diagnosis. Sensitivity and specificity of criteria for GPA were evaluated against MD-diagnosis. Fisher exact test evaluated differences in the frequencies of individual clinical features in GPA versus MPA. RESULTS:Comparing ACR/EULAR criteria against the Ped-EMA algorithm for classifying AAV, more patients were classified as GPA or MPA (n = 396 vs 360, respectively), fewer had GPA (n = 261 vs 288, respectively), more had MPA (n = 135 vs 72, respectively), and fewer GPA cases coclassified as MPA (12% vs 28%, respectively); there were more differences between GPA and MPA in Pediatric Vasculitis Activity Score-defined clinical features (n = 14 vs 10, respectively). When classifying GPA by ACR/EULAR or Ankara criteria, sensitivity (74.5% vs 72.1%, respectively) was comparable, and specificity for ACR/EULAR criteria (93.9% vs 79.9%, respectively) was improved. CONCLUSION:The 2022 ACR/EULAR classification criteria for AAV perform at least as well as previous pediatric criteria and provide categorical MPA criteria where none existed previously; the criteria for GPA and MPA now specifically differentiate each other, with more differences between them in the frequencies of clinical features. Our findings support the preferential use of ACR/EULAR over Ankara criteria for GPA in pediatrics.
OBJECTIVE:To measure healthcare professional-reported conflict and tension across a 10-ward UK children's hospital before and after multimodal interventions. DESIGN AND SETTING:Prospective pre-post single-centre study. PATIENTS:Inpatients with a length of stay ≥5 days. INTERVENTIONS:Five interventions were introduced between 2020 and 2023 centred around a framework to support families and staff: (1) training >400 staff in early recognition and intervention of conflict via the Medical Mediation Foundation, (2) enhancing the use of the Clinical Ethics Advisory Group, (3) introducing palliative care services for all children, (4) promoting safeguarding surgeries and ward-based safeguarding support for staff and (5) developing a senior multidisciplinary hub to advise teams when tension escalated. MAIN OUTCOME MEASURES:Twice weekly on-ward surveys of nursing and medical teams over a 4-week period in 2019 and 2024 RESULTS: Post-intervention, prevalence of children with any reported tensions reduced from 65/153 (42%) to 42/138 (30%) (χ2=4.53, p=0.03) with staff-family conflict predominating. While all sources of tension numerically reduced, reduction in unresolved safeguarding concerns reached statistical significance (18%-3%, χ2=17.6, p<0.0001). Top cited reasons in 2024 for staff-family conflict remained: 'communication breakdown', 'treatment disagreements' and 'unrealistic expectations'; and for staff-staff conflict 'communication breakdown'. For children with ≥2 comorbidities, those with any tensions reduced from 42/76 (55%) in 2019 to 31/82 (38%) in 2024 (χ2=4.84, p=0.028). CONCLUSIONS:We observed an association between multimodal hospital-wide interventions and a reduction in overall tensions. Further studies are needed to assess reproducibility and explore patient and family experience.
OBJECTIVES:Juvenile systemic sclerosis (jSSc) is a rare but severe paediatric rheumatic disease associated with substantial morbidity. Digital vasculopathy is nearly universal and includes Raynaud's phenomenon (RP), digital ulcers (DU) and critical ischemia. Despite advances in adult systemic sclerosis (SSc), progress in jSSc remains limited. This scoping review aimed to identify outcome measures used to assess digital vasculopathy in SSc and jSSc to support core outcome set development for jSSc. METHODS:A scoping review was conducted following PRISMA-ScR guidelines. Medline, Embase, Web of Science, and CENTRAL were searched (1994-2024) for prospective studies reporting outcomes in digital vasculopathy domains (DU, RP, microvascular involvement, telangiectasia). RESULTS:Of 46,002 records, 108 studies were included; 105 (97.2%) involved adults only. Fifty distinct outcome measures were identified, including 35 clinician-reported outcomes (ClinROs) and 15 patient-reported outcomes (PROs). In the DU domain, 12 ClinROs and 5 PROs were identified, with ClinROs most frequently used as primary trial endpoints. In RP, 1 ClinRO and 9 PROs were identified; PROs predominated as primary outcomes. The microvascular domain included 16 ClinROs and no PROs, with instrumental measures used as primary endpoints. In telangiectasia, 6 ClinROs and 1 PRO were identified, rarely used in trials. Only three studies included paediatric patients, and no outcome measures were validated in children. CONCLUSIONS:Outcome measures for digital vasculopathy in SSc are heterogeneous and derived almost entirely from adult studies, with no paediatric-specific validation. Transferability of these measures to children requires formal evaluation. These findings support the need for standardised, validated outcomes for jSSc and inform core outcome set development.
