Objectives This study aimed to test the performance of the “new” American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) criteria, that include anti-nuclear antibodies (ANA) positivity as entry criterion, in juvenile-onset systemic lupus erythematosus (jSLE). Methods Performance of the ACR/EULAR-2019 criteria were compared with Systemic Lupus International Collaborating Clinics (SLICC-2012), using data from children and young people (CYP) in the UK JSLE Cohort Study (n = 482), with the ACR-1997 criteria used as reference standard. An “unselected” cohort of CYP positive for ANA (n = 129) was used to calculate positive/negative predictive values of the criteria. Results At both first and last visit, the number of patients fulfilling the different classification criteria varied significantly (p < 0.001). Sensitivity of SLICC-2012 criteria was higher when compared to ACR/EULAR-2019 at first and last visit (98% vs 94%, first visit, and 98% vs 96%, last visit; p < 0.001), when all available CYP were considered. ACR/EULAR-2019 criteria were more specific when compared to SLICC-2012 (77% vs 67%, first visit, and 81% vs 71%, last visit; p < 0.001). Significant differences between the classification criteria were mainly caused by the variation in ANA positivity across ages. In the “unselected” cohort of ANA positive CYP, ACR/EULAR-2019 criteria produced the highest false positive classification (6/129, 5%) . Conclusion In CYP, ACR/EULAR-2019 criteria are not superior to SLICC-2012 or ACR-1997 criteria. If classification criteria are designed to include CYP and adult populations, paediatric rheumatologists should be included in the consensus and evaluation process, as seemingly minor changes can significantly affect outcomes.
Background Vasculitis can present in many ways and large vessel vascultis is reported rarely to co-present with inflammatory bowel disease. We would like to present two adolescent patients who presented in very similar ways via the gastroenterology team with a seemingly clear diagnosis of inflammatory bowel disease but who were found to have large vessel vasculitis later in their disease journey. The presentation is to raise awareness of this rare co-presentation and to discuss treatment challenges in particular those apparent in adolescent patients crossing the transition bridge. Methods Patient A is a 17 year old boy who has had a long and rocky road to control of his inflammatory bowel disease which presented when he was 2 years old. Histologically it fitted a Crohn’s classification. His journey included moderate response to oral steroids and little to no response to a range of DMARDs, biologics (including infliximab and adalimumab which both had secondary failure) and elemental nutrition, over a period of 12 years. Vedolizumab was introduced this year with almost immediate improvement of gut symptoms, but with ongoing raised inflammatory markers (CRP 79, ESR 86). Incidental investigations of neck pain following the start of vedolizumab revealed significant abnormality in the external carotids, with 70% stenosis. MR angiography confirmed a typical pattern of stenotic large vessel vasculitis. The second patient, B is also 17 and was diagnosed with histological ulcerative colitis aged 14. He has an older brother with IBD but has recently been found to have small bowel disease and is likely therefore to have Crohn’s disease. He is on infliximab 10mg/kg 4 weekly but presented with a 3-month history of high inflammatory markers, malaise towards the end of the 4 week infliximab cycle and drenching night sweats. CT Chest confirmed vasculitis in the thoracic aorta, subclavians and carotids. On PET CT there is mural thickening and no stenosis. Results Patient A presented in paediatric care and B in adult care but because of the seamless rheumatology service and combined MDT with gastroenterology both patients’ care has been widely discussed amongst relevant adult and paediatric teams. Conclusion Large vessel vasculitis might be driving the inflammatory bowel disease in both patients as such the life threatening element of the disease ought to be managed immediately, while ensuring safe transition to between paediatric and adult care. Disclosures S. Rasul None. M. Dockery None. R. Tattersall None. D. Hawley None. S. Maltby None. A. McMahon None.
teams, and after interventions by PDSA3 all night team doctors were taking breaks. Discussion/Conclusion PDSA interventions were successful in increasing staff breaks, however sustainability of results in Junior Doctors will require a culture shift where doctors take personal responsibility for taking breaks. This is well-modelled by the PNPs who effectively arrange cross cover for breaks. A culture shift to improve patient safety overall can be aided by nominating a Junior Doctor break 'Champion'. Encouraging break taking in staff on night shifts will not only increase morale and wellness in the workplace but also contribute to improved patient care.
No abstract
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.