Uvenile Systemic Sclerosis, a Retrospective Epidemiological Study on a French Cohort

Scleroderma is an inflammatory attack of the vessels leading to localized or multisystemic sclerosis. It is a rare autoimmune pathology in pediatrics.

The incidence in pediatrics is very low (about 4 per million according to an American) and therefore the data on the pathology very poor, especially on the therapeutic level.

The proposed immunosuppressive treatments are extrapolated from data in adults. The evolution of connectivity does not seem quite identical to the evolution of adult scleroderma, adaptation of treatments seems judicious. However, data on the evolution under therapy in children are still poor.

Complications related to the pathology, iatrogeny and diagnostic delay are the first causes of mortality from this pathology and deserve to be studied and if possible avoided.

The main hypothesis of the research being to bring together the experiences of the various reference and competence centers in France concerning the clinical presentation, management and follow-up of children with systemic sclerosis.