Juvenile systemic sclerosis (JSSc) is a rare disease of childhood and currently no
international consensus exists with regard to its assessment and treatment. This SHARE
(Single Hub and Access point for paediatric Rheumatology in Europe) initiative, based
on expert opinion informed by the best available evidence, provides recommendations
for the assessment and treatment of patients with JSSc with a view to improving their
outcome. Experts focused attention not only on the skin assessment but also on the
early signs of internal organ involvement whose proper treatment can significantly
affect the long-term outcome. A score for disease severity is proposed in order to
perform a structured assessment of outcome over time but a validation in a wider patient
population is recommended. Finally, a stepwise treatment approach is proposed in order
to unify the standard of care throughout Europe with the aim to reduce morbidity and
mortality in this disease.
