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Multi-centre national audit of juvenile localised scleroderma: describing current UK practice in disease assessment
Hanna Lythgoe1,2,3, Beverley Almeida1,4, Joshua Bennett5
1Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Eaton Road, Liverpool, L12 2AP, UK.
Insights
UK paediatric rheumatology practice for juvenile localised scleroderma (JLS) shows varied assessment but consistent systemic treatment. Improved awareness of Paediatric Rheumatology European Society (PRES) guidelines is needed for standardized JLS care.
Area of Science:
- Paediatric Rheumatology
- Dermatology
- Immunology
Background:
- Juvenile localised scleroderma (JLS) is a chronic autoimmune condition affecting children.
- Current UK practice for JLS assessment and management requires evaluation against established European guidelines.
Purpose of the Study:
- To compare current UK paediatric rheumatology practice for JLS with Paediatric Rheumatology European Society (PRES) scleroderma working party recommendations.
- To identify areas of concordance and divergence in JLS care within the UK.
Main Methods:
- Retrospective data collection from 149 JLS patients across eleven UK centres (04/2015-04/2016).
- Analysis of assessment tools, extracutaneous manifestation screening, and systemic treatment regimens.
Main Results:
- Underutilization of recommended outcome measures like the localised scleroderma cutaneous assessment tool (37.6%).
- Inconsistent screening for uveitis (38.5%) and brain MRI (71.2%) in patients with head involvement.
- Widespread use of systemic treatments (96.0%), with methotrexate as first-line (95.5%) and mycophenolate mofetil as second-line (89.5%) therapy.
Conclusions:
- Significant variation exists in JLS assessment and screening practices in the UK.
- Systemic treatment approaches are relatively consistent, adhering to recommendations.
- Enhanced awareness and implementation of PRES recommendations are crucial for standardizing JLS care; further research is needed for optimal outcome measures and treatments.
Objective:
To describe current United Kingdom practice in assessment and management of patients with juvenile localised scleroderma (JLS) compared to Paediatric Rheumatology European Society (PRES) scleroderma working party recommendations.
Methods:
Patients were included if they were diagnosed with JLS and were under the care of paediatric rheumatology between 04/2015-04/2016. Retrospective data was collected in eleven UK centres using a standardised proforma and collated centrally.
Results:
149 patients were included with a median age of 12.5 years. The outcome measures recommended by the PRES scleroderma working party were not utilised widely. The localised scleroderma cutaneous assessment tool was only used in 37.6% of patients. Screening for extracutaneous manifestations did not meet recommendations that patients with head involvement have regular screening for uveitis and baseline magnetic resonance imaging (MRI) brain: only 38.5% of these patients were ever screened for uveitis; 71.2% had a MRI brain. Systemic treatment with disease-modifying anti-rheumatic drugs (DMARDs) or biologics was widely used (96.0%). In keeping with the recommendations, 95.5% of patients were treated with methotrexate as first-line therapy. 82.6% received systemic corticosteroids and 34.2% of patients required two or more DMARDs/biologics, highlighting the significant treatment burden. Second-line treatment was mycophenolate mofetil in 89.5%.
Conclusion:
There is wide variation in assessment and screening of patients with JLS but a consistent approach to systemic treatment within UK paediatric rheumatology. Improved awareness of PRES recommendations is required to ensure standardised care. As recommendations are based on low level evidence and consensus opinion, further studies are needed to better define outcome measures and treatment regimens for JLS.
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