Vaskulitiden im Kindesalter

Zusammenfassung

Die primären Vaskulitiden sind sehr seltene Erkrankungen im Kindesalter. Die häufigsten Formen sind die Purpura Schönlein-Henoch und das Kawasaki-Sydrom, die einen selbstlimitierenden Verlauf zeigen. Die Ätiopathogenese der Vaskulitiden ist meist noch unklar. Umweltfaktoren wie Infektionen scheinen bei prädisponierten Personen eine verstärkte inflammatorische Antwort auszulösen. Die Klassifikationskriterien der Vaskulitiden im Erwachsenenalter wurden 2008 durch die der europäischen Fachgesellschaft für Kinderrheumatologie (PRES) mit den Vaskulitiden im Kindesalter ergänzt. Die meisten Therapieempfehlungen leiten sich jedoch aus Studienergebnissen aus dem Erwachsenenalter ab. Diese Übersichtsarbeit fasst die aktuellen Klassifikationskriterien, Pathogenesen, klinischen Manifestationen und Therapieprotokolle bei Vaskulitiden im Kindesalter zusammen.

Summary

The primary vasculitides are rare conditions in childhood. The most frequent disease manifestations are Purpura Schoenlein-Henoch and Kawasaki’s syndrome, which usually have a self-limiting course. In the majority of vasculitides, the etiology remains unknown. Environmental factors as well as infections are suspected to trigger an auto-inflammatory response in predisposed individuals. The vasculitides in childhood have been amended to the classification criteria of the vasculitides in adulthood in 2008. Most therapy guidelines for children are adapted from results of studies in adults. This review covers the currrent classifications, pathogenesis, clinical manifestations and therapy recommendations for children.

Schlüsselwörter

Primäre Vaskulitiden - Purpura Schönlein-Henoch - Kawasaki-Sydrom - Klassifikationskriterien

Keywords

Primary vaskulitides - Purpura Schoenlein-Henoch - Kawasaki’s syndrome - classifications

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