Multi-centre national audit of juvenile localised scleroderma: describing current UK practice in disease assessment and management
Abstract 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 diagnos...
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| Format: | Article |
| Language: | English |
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BMC
2018-12-01
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| Series: | Pediatric Rheumatology Online Journal |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s12969-018-0295-0 |
| _version_ | 1819052524227788800 |
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| author | Hanna Lythgoe Beverley Almeida Joshua Bennett Chandrika Bhat Amarpal Bilkhu Mary Brennan Samundeeswari Deepak Pamela Dawson Despina Eleftheriou Kathryn Harrison Daniel Hawley Eleanor Heaf Valentina Leone Ema Long Sarah Maltby Flora McErlane Nadia Rafiq Athimalaipet V. Ramanan Phil Riley Satyapal Rangaraj Giulia Varnier Nick Wilkinson Clare E. Pain |
| author_facet | Hanna Lythgoe Beverley Almeida Joshua Bennett Chandrika Bhat Amarpal Bilkhu Mary Brennan Samundeeswari Deepak Pamela Dawson Despina Eleftheriou Kathryn Harrison Daniel Hawley Eleanor Heaf Valentina Leone Ema Long Sarah Maltby Flora McErlane Nadia Rafiq Athimalaipet V. Ramanan Phil Riley Satyapal Rangaraj Giulia Varnier Nick Wilkinson Clare E. Pain |
| author_sort | Hanna Lythgoe |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-12-21T12:21:12Z |
| format | Article |
| id | doaj.art-29b0dfb5334b4e1fb4895faae4a9294a |
| institution | Directory Open Access Journal |
| issn | 1546-0096 |
| language | English |
| last_indexed | 2024-12-21T12:21:12Z |
| publishDate | 2018-12-01 |
| publisher | BMC |
| record_format | Article |
| series | Pediatric Rheumatology Online Journal |
| spelling | doaj.art-29b0dfb5334b4e1fb4895faae4a9294a2022-12-21T19:04:18ZengBMCPediatric Rheumatology Online Journal1546-00962018-12-011611610.1186/s12969-018-0295-0Multi-centre national audit of juvenile localised scleroderma: describing current UK practice in disease assessment and managementHanna Lythgoe0Beverley Almeida1Joshua Bennett2Chandrika Bhat3Amarpal Bilkhu4Mary Brennan5Samundeeswari Deepak6Pamela Dawson7Despina Eleftheriou8Kathryn Harrison9Daniel Hawley10Eleanor Heaf11Valentina Leone12Ema Long13Sarah Maltby14Flora McErlane15Nadia Rafiq16Athimalaipet V. Ramanan17Phil Riley18Satyapal Rangaraj19Giulia Varnier20Nick Wilkinson21Clare E. Pain22Department of Paediatric Rheumatology, Alder Hey Children’s NHS Foundation TrustDepartment of Paediatric Rheumatology, Alder Hey Children’s NHS Foundation TrustDepartment of Paediatric Rheumatology, Great North Children’s HospitalDepartment of Paediatric Rheumatology, Bristol Royal Hospital for ChildrenDepartment of Paediatric Rheumatology, Royal Hospital for Sick ChildrenDepartment of Paediatric Rheumatology, Royal Hospital for Sick ChildrenDepartment of Rheumatology, Nottingham Children’s Hospital, Nottingham University Hospital NHS TrustDepartment of Rheumatology, Birmingham Children’s HospitalDepartment of Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation TrustDepartment of Rheumatology, Birmingham Children’s HospitalDepartment of Paediatric Rheumatology, Sheffield Children’s HospitalDepartment of Paediatric Rheumatology, Royal Manchester Children’s HospitalDepartment of Paediatric Rheumatology, Leeds Children’s Hospital, Leeds Teaching Hospitals NHS TrustDepartment of Paediatric Rheumatology, Great North Children’s HospitalDepartment of Paediatric Rheumatology, Sheffield Children’s HospitalDepartment of Paediatric Rheumatology, Great North Children’s HospitalDepartment of Paediatric Rheumatology, Leeds Children’s Hospital, Leeds Teaching Hospitals NHS TrustDepartment of Paediatric Rheumatology, Bristol Royal Hospital for Children & Bristol Medical School, University of BristolDepartment of Paediatric Rheumatology, Royal Manchester Children’s HospitalDepartment of Rheumatology, Nottingham Children’s Hospital, Nottingham University Hospital NHS TrustDepartment of Rheumatology and Chronic Pain, Evelina London Children’s Hospital, Guy’s and St Thomas’s NHS Foundation TrustDepartment of Rheumatology and Chronic Pain, Evelina London Children’s Hospital, Guy’s and St Thomas’s NHS Foundation TrustDepartment of Paediatric Rheumatology, Alder Hey Children’s NHS Foundation TrustAbstract 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.http://link.springer.com/article/10.1186/s12969-018-0295-0Juvenile localised sclerodermaMorphoeaPaediatricUnited KingdomAssessmentManagement |
| spellingShingle | Hanna Lythgoe Beverley Almeida Joshua Bennett Chandrika Bhat Amarpal Bilkhu Mary Brennan Samundeeswari Deepak Pamela Dawson Despina Eleftheriou Kathryn Harrison Daniel Hawley Eleanor Heaf Valentina Leone Ema Long Sarah Maltby Flora McErlane Nadia Rafiq Athimalaipet V. Ramanan Phil Riley Satyapal Rangaraj Giulia Varnier Nick Wilkinson Clare E. Pain Multi-centre national audit of juvenile localised scleroderma: describing current UK practice in disease assessment and management Pediatric Rheumatology Online Journal Juvenile localised scleroderma Morphoea Paediatric United Kingdom Assessment Management |
| title | Multi-centre national audit of juvenile localised scleroderma: describing current UK practice in disease assessment and management |
| title_full | Multi-centre national audit of juvenile localised scleroderma: describing current UK practice in disease assessment and management |
| title_fullStr | Multi-centre national audit of juvenile localised scleroderma: describing current UK practice in disease assessment and management |
| title_full_unstemmed | Multi-centre national audit of juvenile localised scleroderma: describing current UK practice in disease assessment and management |
| title_short | Multi-centre national audit of juvenile localised scleroderma: describing current UK practice in disease assessment and management |
| title_sort | multi centre national audit of juvenile localised scleroderma describing current uk practice in disease assessment and management |
| topic | Juvenile localised scleroderma Morphoea Paediatric United Kingdom Assessment Management |
| url | http://link.springer.com/article/10.1186/s12969-018-0295-0 |
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