Ätiologie und Pathogenese des juvenilen systemischen Lupus erythematodes

Zusammenfassung

Der Systemische Lupus erythematodes (SLE) ist eine systemische Autoimmunerkrankung charakterisiert durch die Produktion von zahlreichen Autoantikörpern, insbesondere antinukleären Antikörpern und Antikörper gegen Doppelstrang-DNS, mit episodischen, schubartigen Krankheitsmanifestationen. Die Pathogenese ist gekennzeichnet durch eine Entzündung, die von Autoantikörpern, Immunkomplexen, aktivierten und autoreaktiven Lymphozyten geprägt und durch Ablagerung von Immunkomplexen, Komplementumsatz und zellulären Infiltraten in Geweben und Gefäßen charakterisiert ist. Eine Vielzahl von ätiologischen Faktoren wurde beschrieben: genetische Faktoren, insbesondere mit immunologisch regulativen Eigenschaften, immunologische Mechanismen und exogene Faktoren, zu denen neben Infektionen, Hormonen und UV-Strahlen auch Medikamente zu zählen sind. An der Immunpathogenese beteiligt sind B- und T-Zellen, Makrophagen und dendritische Zellen, Zytokine, Fc-Rezeptoren, Apoptosemechanismen, Immunkomplexe und das Komplementsystem. Die vorliegende Übersicht gibt einen Einblick in die ätiologisch und pathogenetisch bedeutsamen Faktoren beim systemischen Lupus erythematodes.

Abstract

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterised by numerous autoantibodies, especially antinuclear antibodies and antibodies directed to double strand DNA. The heterogeneity of the clinical manifestations reflects the complexity of the disease pathogenesis. The pathogenesis is charcterised by an inflammatory process involving autoantibodies, and activated and autoreactive lymphocytes and deposits of immune complexes, complement and vasclar and parenchymal cellular infiltrates in numerous tissues. The aethiology is unclear but enviromental factors including sex hormones, ultraviolet light exposure and infections are of importance as well as a genetic predisposition with genetic polymorphisms in immunoglobulin Fc receptor genes, cytokine genes, complement genes, those genes that regulate apoptosis and HLA genes. This article reviews the current concepts of the pathogenesis of SLE.

Schlüsselwörter

juveniler systemischer Lupus erythematodes - Pathogenese - Autoimmunität

Key words

juvenile systemic lupus erythematosus - pathogenesis - autoimmunity

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