Consensus-based recommendations for the management of juvenile localised scleroderma

Area of Science:

• Paediatric Rheumatology
• Dermatology
• Autoimmune Diseases

Background:

• Juvenile localised scleroderma (JLS) is a rare paediatric rheumatic disease (PRD) associated with significant morbidity.
• Current management of JLS often relies on physician experience due to a lack of evidence-based guidelines.
• The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative seeks to standardize care for PRDs.

Purpose of the Study:

• To develop evidence-informed consensus recommendations for the assessment and treatment of JLS in children and young adults.
• To establish a European standard of care for JLS management.
• To address the scarcity of evidence-based guidelines in JLS management.

Main Methods:

• An evidence-informed consensus process guided by European League Against Rheumatism (EULAR) standard operating procedures.
• Formation of a committee of 15 experienced paediatric rheumatologists and 2 fellows.
• Systematic literature review, online expert surveys, and nominal group technique consensus meetings.

Main Results:

• One overarching principle, 10 assessment recommendations, and 6 therapy recommendations were accepted with ≥80% expert agreement.
• Recommendations cover assessment of skin and extracutaneous involvement.
• Suggested treatment pathways for JLS were established.

Conclusions:

• The SHARE initiative successfully formulated evidence-based recommendations for JLS assessment and treatment.
• These guidelines provide a much-needed standard of care for paediatric rheumatologists managing JLS patients in Europe.
• The consensus process ensures expert agreement on best practices for JLS management.