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Recent advances in the management of juvenile systemic sclerosis
Francesco Zulian1, Marta Balzarin1, Carolina Birolo1
1a Pediatric Rheumatology Unit, Department for Woman and Child Health , University of Padua , Padua , Italy.
Insights
Juvenile Systemic Sclerosis (JSSc) is a severe pediatric condition requiring early diagnosis and management. Recent advances in classification and assessment tools like J4S aid clinical practice and research, though effective treatments remain limited.
Area of Science:
- Pediatric Rheumatology
- Connective Tissue Diseases
Background:
- Juvenile Systemic Sclerosis (JSSc) is a severe multi-systemic connective tissue disease in children.
- High morbidity and mortality rates necessitate early diagnosis, assessment, and treatment.
Purpose of the Study:
- To review recent advances in the classification and general management of JSSc.
- To highlight new assessment tools and their impact on clinical practice and research.
Main Methods:
- Literature search on JSSc classification and management.
- Incorporation of authors' clinical experience.
Main Results:
- New classification criteria and assessment tools, such as the J4S score, are proposed.
- Novel techniques for internal organ assessment are discussed.
- Current treatments show limited efficacy in preventing complications but may arrest disease progression.
Conclusions:
- Advances in classification and assessment tools are crucial for managing JSSc.
- Effective treatments for JSSc remain a significant challenge, with adult-based therapies showing some promise.
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.
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