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Recent Advances in Systemic Scleroderma in Childhood

Tamas Constantin, Gyorgyi Zoltan, Ivan Foeldvari, Peter Weiser, Andras Szabo.

Abstract
Systemic sclerosis (SSc; scleroderma) is a systemic autoimmune disease characterised by diverse skin and internal organ involvement. The underlying pathology consists of fibrosis caused by fibroblast dysfunction and small vessel vasculopathy. It is much rarer in childhood, meaning 1 in 1 million children, approximately 3-10% of the adult incidence [1, 2]. Only one tenth of juvenile scleroderma (jSSc) has disease onset before 16 years, patients under the age of 10 have equal distribution of males and females. Over 10 years of age, the female predominance as in adult patients, is evolving [3]. The currently published data suggest a dominance of the diffuse subset in children [3]. Pediatric patients have less pulmonary and gastroesophageal involvement and the CREST-syndrome is not observed in the pediatric population [3]. Pediatric patients have better survival rates compared to adults, the 5-year survival was found approximately 93-95% [4, 5]. In the following we attempt to review the hallmarks of this heterogenous disease concentrating on novelties, new results and recommendations.

Key words: advances, systemic sclerosis



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