Juvenile Systemic Sclerosis (jSSc) is an orphan diseases. The incidence is approximately 0.27 per million children. The paediatric rheumatologist pioneered in the establishment of new classification of juvenile scleroderma, which requires only one major criteria and two minor criteria. Regarding presentation of the disease in juvenile age range, they are two large cohorts published. The organ involvement pattern at the time of onset differs from the adults with Systemc Sclerosis (SSc). The mean age at onset is around 8 years. In jSSc patients at pediatric age the diffuse subset dominates and there is a very low occurrence of anticentromere antibodies. The currently running prospective juvenile scleroderma incpetion cohort project (www.juvenile-scleroderma.com), where patient data is collected with a standardized assessment protocoll prospectively, 74% of the patients have diffuse subtype too. Regarding the outcome of the juvenile patients into the adulthood, they are three publications, which show the change of pattern of the organ involvement, which could reflect a survival bias. At the adult age the subset distribution between juvenile onset or young adult onset patients does not differ anymore. The survival of the pediatric patients at 5 years is around 90 to 95%, which is significantly better than in adulthood for the same kind mostly diffuse patient population.
Disclosure of Interest I. Foeldvari Consultant for: chugai, novartis, pfizer,