Childhood Systemic Sclerosis

Francesco Zulian; Giorgia Martini


Curr Opin Rheumatol. 2007;19(6):592-597. 

In This Article


Purpose of review: Juvenile systemic sclerosis has a variety of clinical manifestations, sometimes different from the adult form. Early recognition, proper classification and treatment may improve the long-term outcome.
Recent findings: A large multicenter study coordinated by the Pediatric Rheumatology European Society has yielded important information on the epidemiology and clinical manifestations of systemic sclerosis in childhood. An ad-hoc Committee on Classification Criteria for Juvenile Systemic Sclerosis developed the new classification criteria to help improve patient care by enabling earlier, more definite diagnoses and standardizing the conduct of clinical, epidemiologic, and outcome research for this rare disease. The overall outcome of children with systemic sclerosis is better than in adults but, in those cases with a fatal course, disease progression is rapid and an early involvement of internal organs is associated with poor outcome.
Summary: Studies over the past few years have highlighted the peculiar clinical features and the better outcome of juvenile systemic sclerosis compared with the adult form and propose new pediatric classification criteria. Efforts have recently been made to address the definition of evidence-based recommendations for the treatment of adult and pediatric onset systemic sclerosis.


Juvenile systemic sclerosis (JSSc) is a chronic multisystemic connective tissue disease characterized by symmetrical thickening and hardening of the skin, associated with fibrous changes in internal organs. Although rare in children, it represents one of the most severe rheumatic conditions in pediatric rheumatology practice.

In the past few years, a few published papers have characterized the clinical features of patients with JSSc based on experiences with large cohorts of patients. One publication on pediatric-specific classification criteria was also recently published.

In this paper, we review these important studies along with some new developments on disease monitoring, treatment and long-term outcome. Although many of these studies focus on adult onset systemic sclerosis (SSc), the recommendations may be applicable to children with modifications.


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