Rheumatology Medical News about pediatric systemic sclerosis;developments

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Current developments in pediatric systemic sclerosis
Current Rheumatology Reports, 03/25/09

Foeldvari I et al. - This review focuses on clinical characteristics and outcomes of juvenile systemic sclerosis (jSSc) in childhood and on the clinical presentation and outcome of jSSc patients in adult cohorts of SSc patients. The specific clinical features of jSSc are compared with adult-onset systemic sclerosis.

jSSc, an orphan disease occurs in children at approx. age of 8 yrs, and 90% of pediatric pts have a diffuse subset
Organ involvement of pediatric pts differs from adult pts
Survival rates are significantly better in pediatric pts
Most pts who die in the first 5 yrs have a diffuse subset, that shifts when pediatric pts are reviewed in adult cohorts
In adult cohort only around 40% of pts have diffuse subset
The pediatric-onset pts still have a low prevalence of anticentromere Abs
A larger group of pts have overlap features in jSSc vs adult cohorts
There are also noticeable differences in organ involvement

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