Update on the Systemic Treatment of Pediatric Localized Scleroderma

Area of Science:

• Pediatric Rheumatology
• Dermatology
• Immunology

Background:

• Juvenile localized scleroderma (jLS) is a rare condition causing disfigurement and orthopedic issues.
• Current guidelines emphasize the Localized Scleroderma Cutaneous Assessment Tool for monitoring.
• Systemic treatment is crucial for managing progressive or severe jLS cases.

Purpose of the Study:

• To review and summarize the current systemic treatment strategies for juvenile localized scleroderma.
• To highlight the efficacy and sequencing of disease-modifying antirheumatic drugs in jLS.
• To discuss potential future therapeutic avenues based on adult scleroderma research.

Main Methods:

• Review of recent publications and clinical trial data on jLS systemic therapy.
• Analysis of treatment recommendations for pediatric localized scleroderma.
• Extrapolation of findings from adult systemic sclerosis and fibrosis treatment studies.

Main Results:

• Methotrexate is the established first-line systemic treatment for jLS, supported by controlled trials.
• Methotrexate demonstrates superior efficacy compared to phototherapy.
• Alternative agents like mycophenolate mofetil, tocilizumab, and abatacept are effective for methotrexate intolerance or nonresponse.

Conclusions:

• Systemic therapy is indicated for jLS involving joints, cosmetic disfigurement, or orthopedic concerns.
• Methotrexate is the cornerstone of systemic jLS treatment.
• Emerging therapies and approaches from adult scleroderma research hold promise for future jLS management.