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.