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Pädiatrie up2date 2012; 07(02): 191-212
DOI: 10.1055/s-0032-1309806
Rheumatologie
© Georg Thieme Verlag KG Stuttgart · New York

Primäre Vaskulitiden im Kindesalter

Wilma Mannhardt-Laakmann
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Publication History

Publication Date:
21 June 2012 (online)

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Einleitung

Vaskulitiden gehören zu den komplexen entzündlichen Multisystemerkrankungen. Die Vaskulitis wird als Entzündungsgeschehen in der Gefäßwand definiert. Pathologisch-anatomisch werden die Zellwände durch die Entzündungsreaktion (Exsudation mononukleärer, eosinophiler oder neutrophiler Zellen) destruiert, was histologisch als fibrinoide Nekrose sichtbar wird (nekrotisierende Vaskulitis) [1] [2]. Die Vaskulitis kann auf ein Organ beschränkt auftreten, häufiger jedoch findet sich eine generalisierte Verteilung. Je nach Kalibergröße des betroffenen Gefäßes (Abb. [1]) finden sich fokale Läsionen mit dem Risiko der Ausbildung von Aneurysmata; häufiger sind es segmentale Läsionen, die zur Gefäßstenosierung oder Okklusion und Infarzierung der im Stromgebiet liegenden Organe führen können [1].

Zoom Image
Abb. 1 Einteilung der systemischen Vaskulitiden nach Gefäßgrösse (nach Jennette JC et al., Arthritis Rheum 1994; 37: 187 – 192).

Die Gefäßentzündung kann primär oder auch im Rahmen einer Grunderkankung auftreten. Die für das Kindesalter typischen Vaskulitiden, Purpura Schönlein-Henoch und Kawasaki-Syndrom, lassen sich im Wesentlichen aufgrund der Haut- und Begleitsymptome klinisch diagnostizieren.

Alle anderen primären Vaskulitiden treten im Kindesalter sehr selten auf. Es ist daher wichtig, alle Erkrankungen zu kennen, die eine Vaskulitis imitieren können, und sie vor einer eventuell invasiven Therapie auszuschließen (Tab. [1]).

Tabelle 1

Differenzialdiagnose bei sekundären Vaskulitiden [2].

Infektionen

Hepatitis A/B/C

HIV

HSV

Mykobakterien

Pilz-Aneurysmen

bakterielle Endokarditis

Protozoen

Medikamente / Toxine

Antibiotika (Penicilline, Sulfonamide)

Thiazide

Cumarin-Derivate

NSAID

Hydantoine

Hyperkoagulopathie

Antiphospholipid-Syndrom

Malignome

Lymphome

Leukämien

Vasospasmen

Vasokonstriktions-Syndrom

posteriore Leukenzephalopathie (beide reversibel)

Immundefekte

CVID

HLA-Klasse-I-Defekt

angeborene Störungen

Marfan-Syndrom

Ehlers-Danlos-Syndrom

Pseudoxanthoma elasticum

Neurofibromatose Typ 1

Fibromuskuläre Dysplasie

Moyamoya

Inflammationssyndrom

Sarkoidose

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