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Sklerodermie im Kindes- und Jugendalter

Neue Aspekte zu Klassifikation, Ätiologie und Therapie

Scleroderma in childhood and adolescence

New aspects on classification, etiology and therapy

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Zusammenfassung

Die Sklerodermie wird nach klinischen Gesichtspunkten im Wesentlichen in 2 Ausprägungsarten unterschieden: die lokalisierte Sklerodermie und die systemische Sklerodermie. Da sich diese didaktische Trennung im Patienten nicht immer abbildet, haben verschiedene Gruppen in den letzten Jahren versucht, neue klinische Klassifikationssysteme zu erarbeiten. Kürzliche Erkenntnisse zu Ätiologie und Pathophysiologie bilden sich darin aber noch unzureichend ab. Der folgende Beitrag trägt diesem Umstand Rechnung, indem er die klinische Beschreibung der Sklerodermie mit den neuesten Erkenntnissen vor allem auch zu molekularer Pathophysiologie zusammenfügt und moderne Aspekte der Therapiestrategien aufzeigt.

Abstract

From a clinical point of view scleroderma is classified as either localized scleroderma or systemic scleroderma. However, as this didactic separation does not always reflect the clinical picture in patients, various groups have attempted to make new classification methods. This current review focuses on the clinical presentation in this respect, recently developed aspects of etiology and molecular pathogenesis in addition to modern aspects of therapeutic strategies.

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Danksagung

Der Autor dankt PD Dr. Meinrad Beer, Institut für Röntgendiagnostik der Universität Würzburg, für die Überlassung der Bilder zu Abb. 5 e, d, f, g der radiologischen Diagnostik.

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  1. Pädiatrische Rheumatologie, Immunologie, Infektiologie und Gastroenterologie, Universitäts-Kinderklinik Würzburg, Josef-Schneider-Straße 2, 97080, Würzburg, Deutschland

    H.J. Girschick

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Girschick, H. Sklerodermie im Kindes- und Jugendalter. Z. Rheumatol. 67, 128–136 (2008). https://doi.org/10.1007/s00393-007-0253-6

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