IgG4-assoziierte Erkrankungen

ZUSAMMENFASSUNG

IgG4-assoziierte Erkrankungen sind fibrosierende entzündliche Systemerkrankungen, die nahezu jedes Organsystem betreffen können. Sie betreffen meistens Männer in einem Alter über 50 Jahre. Zu den Krankheitsmanifestationen zählen u. a. Autoimmunpankreatitis, Lymphadenopathien, retroperitoneale Fibrose, entzündliche Orbitopathie sowie Beteiligungen der Speichel- und Tränendrüsen. Diese können gemeinsam oder isoliert auftreten. In der Diagnostik sind eine histologische Untersuchung sowie die Bestimmung des IgG4 im Serum wichtig. Maligne und andere entzündliche Erkrankungen müssen ausgeschlossen werden. IgG4-assoziierte Erkrankungen sprechen gut auf Glukokortikosteroide an.

ABSTRACT

IgG4-related diseases are fibro-inflammatory diseases that can affect almost every organ. Disease manifestations include autoimmune pancreatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory orbitopathy and involvement of salivary and lacrimal glands. They can occur either isolated or in combination. The measurement of serum IgG4 and a histology of involved organs is important in the diagnostics of IgG4-related diseases. Malignancies and other inflammatory diseases have to be excluded. Glucocorticosteroids lead to rapid improvement of clinical manifestations.

Schlüsselwörter

IgG4 - IgG4-assoziierte Erkrankungen - Periaortitis - Autoimmunpankreatitis

Keywords

IgG4 - IgG4-related disease - periaortitis - autoimmune pancreatitis

Publikationsverlauf

Artikel online veröffentlicht:
07. September 2022

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