Update on pediatric systemic sclerosis: similarities and differences from adult disease

Abstract

Purpose of review: Aim of the review is to summarize the current knowledge about clinical presentation and prognosis of patients with juvenile onset systemic sclerosis and compare it with that of those with adult onset systemic sclerosis.

Recent findings: Juvenile onset systemic sclerosis and adult onset systemic sclerosis have a different subtype and organ involvement pattern of the juvenile onset patients in the pediatric age is compared with that of adult patients. The juvenile onset patients have 90% diffuse subset, despite a 5-year survival of around 90%. The organ involvement pattern in both the groups becomes equal over the disease duration, but renal involvement is rare. The subset pattern equalizes over time too; the adult-aged juvenile onset patients have the same disease subset distribution, with around 40% diffuse subset patients, as the young adult onset patients, and this presents a survival bias presumably. The survival even after 20-year disease course seems more favourable for the juvenile onset patients.

Summary: Currently we are looking at two time windows: one at the first 5 years of the disease and the second at 20 years after the onset. We do not have the data on why and when the subset distribution changes occur.