Development of minimum standards of care for juvenile localized scleroderma

作者：Constantin Tamas^*; Foeldvari Ivan; Pain Clare E; Palinkas Annamaria; Hoeger Peter; Moll Monika; Nemkova Dana; Weibel Lisa; Laczkovszki Melinda; Clements Philip; Torok Kathryn S

来源：European Journal of Pediatrics, 2018, 177(7): 961-977.

DOI：10.1007/s00431-018-3144-8

• Localized scleroderma
• Diagnosis
• Treatment
• Guideline

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.
Conclusion: Management of jLS is varied. These recommendations should help provide standardization of assessment and care for those with this rare and potentially debilitating condition.
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 [1]. _ 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.

• 出版日期2018-7