Komplementdefekte

Zusammenfassung

Das Komplementsystem besteht aus einer Vielzahl von Einzelproteinen und wird als Teil des unspezifischen, angeborenen humoralen Immunsystems angesehen. Hauptaufgaben sind die Beteiligung an der Bakterienabwehr, der Elimination von Immunkomplexen und Entzündungsreaktionen. Genetische Defekte sind bei fast allen Komponenten beschrieben. Häufigster Defekt ist das hereditäre Angioödem (HAE), verursacht durch Fehlen oder Dysfunktion des C1-Inhibitors. Es muss differenzialdiagnostisch vom allergischen Quincke-Ödem abgegrenzt werden. Andere Defekte fallen durch Neigung zu schweren bakteriellen Infektionen, insbesondere Meningokokkeninfektionen, oder durch autoimmune oder autoimmunähnliche Erkrankungen auf.

Summary

Complement system is composed of several individual components. It is part of non-specific, innate humoral immunity. It contributes to antibacterial defense, elimination of circulating immune complexes and inflammatory responses. Genetic defects have been described for almost all components of the complement system. The most frequent defect is the hereditary angioedema HAE, caused by absence or dysfunction of the C1-Inhibitor (C1-INH) molecule. HAE is an important differential diagnosis of the allergic Quincke edema. Deficiencies of the other complement proteins present with increased susceptibility to severe bacterial infections, especially meningococcal infections, or with autoimmune or autoimmune-like diseases.

Schlüsselwörter

Komplementdefekte - hereditäres Angioödem - Meningokokkeninfektionen - Autoimmunerkrankungen

Keywords

Complement deficiencies - hereditary angioedema - meningococcal infections - autoimmune disorders

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