United Kingdom survey of current management of juvenile localized scleroderma.
OBJECTIVE
Juvenile localized scleroderma (JLS) is a rare condition that is often difficult to assess and for which a variety of monitoring tools have been described. We aimed to describe how monitoring tools are used and perceived by clinicians in the UK, to ascertain treatments used for JLS and to provide a description of transition arrangements to adult care.


METHODS
An e-survey of UK paediatric rheumatologists and dermatologists managing children and young people (CYP) with JLS was distributed using the national organisations representing these clinician groups. We asked respondents for their views and experience using 15 JLS monitoring tools, about transition services and about treatments used.


RESULTS
Thirty-five dermatologists and 13 paediatric rheumatologists responded. Paediatric rheumatologists managed more CYP with JLS than dermatologists (median 16-20 and 3, respectively). Transition arrangements were reported by 43% of dermatologists and 91% of paediatric rheumatologists. Medical photography was the most frequently regularly used monitoring tool (73% respondents). The modified Rodnan skin score was the skin score used most commonly: 33% of paediatric rheumatologists and 3% of dermatologists reported using this tool frequently. Topical treatments and ultraviolet light were used by 49-80% of dermatologists and 0-8% paediatric rheumatologists. Biologic drugs and CYC were used by 0-3% of dermatologists and 31-46% of paediatric rheumatologists.


CONCLUSIONS
How monitoring tools are accessed, used and perceived by paediatric rheumatologists and dermatologists in the UK varies between and within clinician groups, as do treatment prescribing patterns and transition arrangements. These differences will impact on the feasibility of conducting multicentre clinical trials and on standardising clinical care.
Department of Paediatric Rheumatology, Sheffield Children's NHS Foundation Trust, Sheffield, Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, Department of Dermatology, Nottingham University Hospitals NHS Trust, Nottingham, Department of Dermatology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle University, and Department of Paediatric Rheumatology, Great North Children's Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK. dhawley@doctors.org.uk.
Daniel P
DP
Hawley
Department of Paediatric Rheumatology, Sheffield Children's NHS Foundation Trust, Sheffield, Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, Department of Dermatology, Nottingham University Hospitals NHS Trust, Nottingham, Department of Dermatology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle University, and Department of Paediatric Rheumatology, Great North Children's Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.
Clare E
CE
Pain
Department of Paediatric Rheumatology, Sheffield Children's NHS Foundation Trust, Sheffield, Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, Department of Dermatology, Nottingham University Hospitals NHS Trust, Nottingham, Department of Dermatology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle University, and Department of Paediatric Rheumatology, Great North Children's Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.
Eileen M
EM
Baildam
Department of Paediatric Rheumatology, Sheffield Children's NHS Foundation Trust, Sheffield, Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, Department of Dermatology, Nottingham University Hospitals NHS Trust, Nottingham, Department of Dermatology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle University, and Department of Paediatric Rheumatology, Great North Children's Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.
Ruth
R
Murphy
Department of Paediatric Rheumatology, Sheffield Children's NHS Foundation Trust, Sheffield, Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, Department of Dermatology, Nottingham University Hospitals NHS Trust, Nottingham, Department of Dermatology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle University, and Department of Paediatric Rheumatology, Great North Children's Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.
Aileen E M
AE
Taylor
Department of Paediatric Rheumatology, Sheffield Children's NHS Foundation Trust, Sheffield, Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, Department of Dermatology, Nottingham University Hospitals NHS Trust, Nottingham, Department of Dermatology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle University, and Department of Paediatric Rheumatology, Great North Children's Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK. Department of Paediatric Rheumatology, Sheffield Children's NHS Foundation Trust, Sheffield, Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, Department of Dermatology, Nottingham University Hospitals NHS Trust, Nottingham, Department of Dermatology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle University, and Department of Paediatric Rheumatology, Great North Children's Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.
Helen E
HE
Foster
D000287:Administration, Topical; D000293:Adolescent; D002648:Child; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use*; D019538:Health Care Surveys; D006801:Humans; D010372:Pediatrics; D010818:Practice Patterns, Physicians'; D012594:Scleroderma, Localized / Q000175:diagnosis* / Q000188:drug therapy / Q000628:therapy*; D014467:Ultraviolet Therapy
D005938:Glucocorticoids
access to care; laser Doppler; localized scleroderma; magnetic resonance imaging; paediatric; photography; skin; skin score; thermography; ultrasound