Current developments in pediatric systemic sclerosis.

Juvenile systemic sclerosis is an orphan disease with an incidence of around 0.05 per 100,000 children. The mean age of onset is approximately 8 years, and 90% of pediatric patients have a diffuse subset. The organ involvement of pediatric patients differs from adult patients. Survival rates are significantly better in pediatric patients than in adult patients. Most patients who die in the first 5 years of the disease have a diffuse subset. Interestingly, the disease subset shifts when pediatric patients are reviewed in adult cohorts of systemic sclerosis patients, in which only around 40% of patients have diffuse subset. The pediatric-onset patients still have a low prevalence of anticentromere antibodies. There is a larger group of patients with overlap features in juvenile systemic sclerosis than in adult cohorts. There are also noticeable differences in organ involvement.