Background In 2012, a European initiative called Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile Scleroderma in its two variety, localized scleroderma (JLS) and systemic sclerosis (JSS) is a rare disease within the group of paediatric rheumatic diseases (PRDs) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. Consequently, treatment regimens differ throughout Europe.
Objectives To provide recommendations for diagnosis and treatment of both JLS and JSS.
Methods Recommendations were developed by an evidence-informed consensus process using the EULAR standard operating procedures. A committee was constituted, consisting of 16 experienced paediatric rheumatologists, mainly from Europe. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using nominal group technique. Recommendations were accepted if >80% agreement was reached.
Results In total, 1 overarching principle, 17 recommendations on diagnosis and 13 recommendations on therapy were accepted with >80% agreement among experts. Topics covered include assessment of skin and major organ involvement and suggested treatment pathways.
Conclusions The SHARE initiative aims to identify best practices for treatment of patients suffering from PRD. Within this remit, recommendations for the diagnosis and treatment of JLS and JSS have been formulated by an evidence informed consensus process to produce a standard of care throughout Europe.
Disclosure of Interest None declared