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; classification; outcome measures; severity score; systemic sclerosis; treatment.