<p>Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1&#xa0;million children. It has many similarities with the adult-onset disease, but it has its own specific characteristics regarding organ involvement and antibody pattern. Approximately 70% of patients have the diffuse subtype. The 2013 classification criteria of the European Alliance of Associations for Rheumatology (EULAR) can also be applied to jSSc. In the assessment of patients, the specific pediatric issues must be considered. Treatment is guided by published pediatric and adult recommendations.</p>

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Juvenile systemische Sklerose

  • Ivan Foeldvari

摘要

Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 million children. It has many similarities with the adult-onset disease, but it has its own specific characteristics regarding organ involvement and antibody pattern. Approximately 70% of patients have the diffuse subtype. The 2013 classification criteria of the European Alliance of Associations for Rheumatology (EULAR) can also be applied to jSSc. In the assessment of patients, the specific pediatric issues must be considered. Treatment is guided by published pediatric and adult recommendations.