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Immunologische Grundlagen der IgG4-RD

Immunological principles of IgG4 related diseases

  • Leitthema
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Zusammenfassung

IgG4 bindet nicht wie die anderen IgG-Subklassen an niedrigaffine Fc-Rezeptoren und aktiviert nicht die klassische Komplementkaskade. Es ist zudem instabil und kann spontan in 2 Hemimoleküle zerfallen. Auch wenn diese Daten für eine immunsuppressive Rolle des IgG4 sprechen, gibt es andererseits wenige Beispiele, in denen IgG4-Antikörper eine immunaktivierende Funktion ausüben. Die immunologische Funktion des IgG4 im Rahmen der IgG4-assoziierten Erkrankungen ist daher noch nicht völlig geklärt. Auch die Auslöser der IgG4-RD (allergisch oder autoimmun) sind noch umstritten. In der Pathophysiologie der Erkrankung spielt die Aktivierung der Th2-Zellen und der regulatorischen T‑Zellen eine wichtige Rolle, die dann über die Produktion von Zytokinen einerseits zur Bildung des IgG4 und andererseits zur Gewebefibrosierung beitragen.

Abstract

In contrast to the other IgG subclasses, IgG4 does not bind to low affinity Fc receptors or activate the classical complement pathway. In addition, it is unstable and can dissociate into two hemimolecules; therefore, IgG4 most likely has an immunosuppressive role. On the other hand, there a few examples of an immunostimulatory role of IgG4 antibodies; therefore, the function of IgG4 in IgG4 related diseases is not yet entirely clear. The trigger factors of IgG4 related diseases (allergic or autoimmune) are still under debate. The activation of T helper (Th) 2 and regulatory T cells has been shown to be important in the pathophysiology of IgG4 related diseases as they produce cytokines which contribute to the formation of IgG4 and to fibrosis of various tissues.

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  1. Klinik für Immunologie und Rheumatologie, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    T. Witte

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P. Lamprecht, Lübeck

F. Moosig, Neumünster

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Witte, T. Immunologische Grundlagen der IgG4-RD. Z Rheumatol 75, 660–665 (2016). https://doi.org/10.1007/s00393-016-0123-1

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  • DOI: https://doi.org/10.1007/s00393-016-0123-1

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