Recent advances in the management of juvenile systemic sclerosis

ABSTRACT

Introduction: Juvenile Systemic Sclerosis (JSSc) is one of the most severe multi-systemic connective tissue conditions encountered in pediatric rheumatology. Due to its high morbidity and mortality rate, early diagnosis, proper assessment and effective treatment are crucial.

Areas covered: In this review, we will focus on the recent advances on the classification and general management of JSSc based on the literature search and on the Authors’ experience.

Expert commentary: Classification criteria for the pediatric forms of systemic sclerosis, the new proposed assessment tools, such as the juvenile systemic sclerosis severity score, named J4S, and new techniques for the internal organs assessment, will facilitate both daily practice and research projects. Unfortunately, no known drugs seem to be effective in preventing the development of disease complications although a few treatments, undertaken according to experience in adults, have shown some effects in arresting the disease progression.

KEYWORDS:

• Scleroderma
• systemic sclerosis
• classification
• outcome measures
• severity score
• treatment

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Additional information

Funding

This paper was not funded.