Aktuelle Rheumatologie 2012; 37(05): 288-294
DOI: 10.1055/s-0032-1314831
Übersichtsarbeit
© Georg Thieme Verlag KG Stuttgart · New York

Die funktionellen Besonderheiten von IgG4 und die IgG4-assoziierten Krankheiten aus Sicht des Immunologen

The Functional Properties of IgG4 and the IgG4-Related Diseases – The Immunologist’s Point of View
B. Mueller-Hilke
1   Institute of Immunology, University of Rostock, Rostock
,
M. Seidl
2   Institut für Pathologie, Universitätsklinikum Freiburg, Freiburg
,
H. H. Peter
3   Centrum für chronische Immundefizienz (CCI), University Medical Center, Freiburg
› Author Affiliations
Further Information

Publication History

Publication Date:
20 June 2012 (online)

Zusammenfassung

Obwohl IgG4 nur einen geringen Anteil der Serum Immunglobuline ausmacht und die immunologischen Effektormechanismen wie Komplementaktivierung und Bindung an Fc-Rezeptoren nur unzureichend vermittelt, mehren sich derzeit die Berichte über IgG4-vermittelte Erkrankungen (IgG4-related diseases; IgG4-RD). Diese stellen eine erst kürzlich neu definierte Krankheitsentität dar, die durch eine diffuse oder noduläre lympho-plasmazelluläre (IgG4+) Gewebsinfiltration mit chronisch-fibrosierender Entzündungsreaktion in verschiedensten Organen gekennzeichnet ist. Serum IgG4 kann, muss aber nicht erhöht sein, häufig lassen sich diverse IgG4-positive Autoantikörper im Serum nachweisen. Wir fassen die Faktoren, die einen Klassenwechsel zum IgG4 begünstigen, sowie die strukturellen Besonderheiten des IgG4 Moleküls zusammen und diskutieren Mechanismen, wie der Fab-Armaustausch die periphere B-Zelltoleranz beeinflusst und die chronische Immunpathologie bei IgG4-RD unterhalten könnte.

Abstract

The IgG4 subclass accounts only for a small amount of total IgG (3–5%) and exhibits poor effector properties with regard to complement and FcRgamma activation. Yet since 10 years an increasing number of reports has defined a new set of IgG4-related diseases (IgG4-RD) characterised by elevated serum IgG4 levels and diffuse or nodular lympho-plasmocytic infiltrates (>40% IgG4+ plasma cells) in various, mainly exocrine organs. The infiltrates are typically associated with a chronic-fibrosing inflammation. While the serum IgG4+ fraction is not always increased it contains an increased proportion of various autoantibodies. Here we review the structural properties of the IgG4 molecule and the factors regulating class-switch to IgG4 and we discuss how the unique property of IgG4 to undergo Fab-arm exchange may influence the peripheral B cell tolerance and impact on the immunopathology of IgG4-RD.

 
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