Development of minimum standards of care for juvenile localized scleroderma
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Juvenile localized scleroderma (jLS), also known as morphea, is an orphan disease. Pediatric guidelines regarding diagnosis, assessment, and management are lacking.
Our objective was to develop minimum standards of care for diagnosis, assessment, and management of jLS. A systematic review was undertaken to establish the pediatric evidence for assessment and monitoring of jLS. An expert panel, including members of the Pediatric Rheumatology European Society (PRES) Scleroderma Working Group, were invited to a consensus meeting where recommendations were developed based on evidence graded by the systematic review and, where evidence was lacking, consensus opinion. A nominal technique was used where 75% consensus was taken as agreement. Recommendations for diagnosis, assessment, and management were developed. Due to a lack of pediatric evidence, these were primarily consensus driven. Careful assessment for extra-cutaneous manifestations including synovitis, brain involvement, and uveitis were key features together with joint assessments between Dermatology and Rheumatology to improve and standardize care.
What is Known:
• Children with juvenile localized scleroderma (jLS) are managed by a number of specialties including pediatric rheumatologists and dermatologists, sometimes in shared clinics. Studies have shown that management varies considerably and that there are notable differences between specialties .
• There is very little published guidance on management of jLS.
What is new:
• These recommendations aim to standardize diagnosis, assessment, and management through review of pediatric evidence and consensus agreement.
• Joint review of patients by both pediatric rheumatologists and dermatologists is recommended.
KeywordsLocalized scleroderma Diagnosis Treatment Guideline
Childhood Arthritis and Rheumatology Alliance
Children’s Dermatology Life Quality Index
Dyspigmentation, Induration, Erythema, Telengiectasias
Disease-modifying anti-rheumatic drug
En coup de saber
Erythrocyte sedimentation rate
European League Against Rheumatism
Juvenile localized scleroderma
Localized Scleroderma Clinical and Ultrasound Study
Localized Scleroderma Cutaneous Assessment Tool
Localized Scleroderma Damage Index
Modified Localized Scleroderma Severity Skin Index
Modified Rodnan Skin Score
Pediatric Rheumatology European Society
- PUVA therapy
Psoralen ultraviolet A
Quality of life
Kathryn S. Torok is senior author. Ivan Foeldvari designed the initiative. Annamária Pálinkás, Melinda Laczkovszki performed the systematic literature review, supervised by Tamás Constantin. Validity assessment of selected papers was done by Tamás Constantin, Ivan Foeldvari, Clare E Pain, Peter Höger, Monika Moll, Dana Nemkova, Lisa Weibel, Kathryn S. Torok. Recommendations were formulated by Tamás Constantin, Ivan Foeldvari and Clare E Pain. The expert committee consisted of Tamás Constantin, Ivan Foeldvari, Clare E Pain, Peter Höger, Monika Moll, Dana Nemkova, Lisa Weibel, Kathryn S. Torok; they completed the online surveys and participated in consensus meeting. Philip Clements assisted in the preparation of the live consensus meeting and facilitated the consensus procedure using nominal group technique. Tamás Constantin, Clare E Pain and Kathryn S. Torok wrote the manuscript, with contribution and approval of all co-authors.
no confidential data were used regarding this work.
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