- To learn about the evolvement of organ involvement and quality of life from patients with juvenile systemic sclerosis
- To improve the care of juvenile systemic sclerosis patients worldwide
The juvenile scleroderma inception cohort project (www.juvenile-scleroderma.com) collects data from juvenile systemic scleroderma (jSSc) patients prospectively with a standardised assessment of the organ systems. We included now over 100 children with jSSc in this cohort. This is the largest prospective cohort of the world. jSSc is an orphan disease, with a prevalence of 3 per 1 000 000 children. Each recruited patient is important for us.
Please tell your treating physician to participate in this cohort.
The juvenile scleroderma inceptions project has the aim to learn more about jSSc; the evolvement of the organ involvement, about the prognosis of the disease and about the factors influencing the prognosis. In this project the responsivness of the outcome measures are assessed, if they are sensible enough to find occurring changes over time. This is a very important issue, as early recognition of an organ involvement helps to guide an intensification of the therapy. As in other autoimmune diseases, there is a feeling, that early intervention, leads to prevention of damage of the organ systems and significantly improves survival. There are currently no controlled studies, which explore the effectivity of certain medications in jSSc, but based on the inception cohort data, clinical trials for licencing medication for jSSc are on the way.
The validation of the paediatric outcome measures for jSSc , which is one an other aim of the project will enable us to conduct prospective therapeutic trials for our paediatric patients more sufficiently.
An other important question is for the paediatric patients and their parents, what is the long term prognosis of patients with jSSc. Currently we have only indirect data from adult patient cohorts, where juvenile onset patients are incorporated. During the longitunidal follow up, we will learn more about the long term prognosis too.
With your help, with each included patient, we will learn more about jSSc and jSSc will be a much more controllable disease in the near future.