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DOI: 10.1055/s-0037-1618172
Update zur juvenilen systemischen Sklerodermie
Juvenile systemic sclerosis – updatePublication History
Publication Date:
26 December 2017 (online)
Zusammenfassung
Die juvenile systemische Sklerodermie (jSSc) ist eine „Orphan“-Erkrankung mit einer Inzidenz von 0,27 bis 0,5 pro eine Million Kinder. Bei bis zu 90 Prozent der Patienten ist ein diffuser Verlauf zu beobachten, dennoch ist die Überlebensrate besser als bei erwachsenen Patienten. Bei langfristigem Verlauf sinkt der Anteil der Patienten mit diffuser Sklerodermie auf 40 Prozent. Die Overlap-Symptomatik scheint einen Überlebensvorteil zu bieten. Die meisten SSc-spezifischen Erfassungsmethoden sind bei der jSSc nicht validiert. Die Therapie beruht auf Erfahrungen aus der Erwachsenenmedizin.
Summary
Juvenile systemic sclerosis is an orphan disease with an incidence of 0.27 to 0.5 in a million children. In up to 90 % of the patients a diffuse course of disease can be noticed. However, the survival rate is higher than in adult patients. In the long-term course the part of patients with a diffuse form of sclerosis decreases to 40 %. The „overlap“ symptomatology seems to offer a survival benefit. Most of the detection methods specific for sclerosis are not validated for the juvenile systemic sclerosis. Therapy is based on the experiences made in adult medicine.
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