O26 Describing current UK practice in assessment and management of juvenile localised scleroderma and consideration in context of PRES working party recommendations

Abstract

Background: There is limited evidence on optimal assessment and treatment regimens for juvenile localised scleroderma (JLS). The Paediatric Rheumatology European Society (PRES) scleroderma working group has produced consensus recommendations for diagnosis, assessment and management of JLS. We aimed to describe current UK practice with regards to assessment and management of JLS and consider these in the context of PRES working party recommendations. Methods: Patients were eligible if they had a diagnosis of JLS and were under the care of a paediatric rheumatologist between April 2015 and April 2016. Data was collected from eleven UK centres using a standardised proforma. Ethical approval was not required as this was an audit against published standards of care. Results: 149 patients were included with a mean age at time of audit of 12.1 years and 7.6 years at time of diagnosis. Patients had a variety of disease subtypes: 67.1% linear scleroderma; 14.8% plaque morphoea; 10.1% generalised morphoea; 1.3% pansclerotic disease; 6.7% mixed disease. 16.1% (24/149) had extracutaneous manifestations including three (2.0%) with uveitis and two (1.3%) with CNS vasculitis. UK practice is not consistently in line with PRES working party recommendations (Table 1). Screening for extracutaneous manifestations was varied. Recommendations include that patients with craniofacial involvement have regular screening for uveitis and an MRI brain. Of patients with craniofacial involvement (n=52), only 38.5% were ever screened for uveitis with only one patient receiving six monthly screening. 37 (71.2%) of these patients had MRI imaging. Standardised outcome measures were only used in 83/149 (55.7%). Systemic immunosuppressive treatment was widely used (96.0%) and met standards of care in all patients. 95.5% received methotrexate as first line therapy. 34.2% required two or more sequential immunosuppressants and 86.0% received systemic corticosteroids highlighting the significant treatment burden. Conclusion: Although there is a consistent approach to systemic treatment within UK paediatric rheumatology, there is wide variation in assessment and screening of JLS patients. Improved awareness of these recommendations is required to ensure standardised care. As these recommendations are based on low level evidence and consensus opinion, further studies are needed to better define outcome and treatment regimens.