IgG4-assoziierte Erkrankungen – Das komplexe Krankheitsbild, Organbeteiligung, Komplikationen

Zusammenfassung

IgG4-assoziierte Erkrankungen sind seltene, entzündlich fibrosierende Systemerkrankungen mit vielgestaltigem Verlauf, die nahezu jedes Organsystem betreffen können. Zu den häufigen Manifestationen gehören eine Autoimmunpankreatitis, Lymphadenopathien, die Retroperitonealfibrose, eine entzündliche Orbitopathie sowie Beteiligungen der Speichel- und Tränendrüsen. Alle Manifestationen können isoliert oder in Kombination auftreten und stellen oft eine diagnostische Herausforderung dar, da sie sowohl maligne als auch andere entzündliche Erkrankungen imitieren können. Mittlerweile wurden 4 Cluster identifiziert, die die Hauptphänotypen darstellen. Komplikationen entstehen durch die progressive Fibrose, die zu irreversiblen Organschäden führen kann, die neben der medikamentösen Therapie auch ein chirurgisches Vorgehen notwendig machen können.

Abstract

IgG4-related diseases are rare, inflammatory fibrosing systemic diseases with a potentially complex course that can affect almost any organ system. Common manifestations include autoimmune pancreatitis, lymphadenopathies, retroperitoneal fibrosis, inflammatory orbitopathy and involvement of the salivary and lacrimal glands. These can occur alone or in combination and often pose a diagnostic challenge as they can mimic both malignant and other inflammatory diseases. Four clusters have been identified that represent the main disease phenotypes. Complications arise from progressive fibrosis, which can lead to irreversible organ damage that may require surgery in addition to immunomodulatory therapy.

Schlüsselwörter

IgG4 - Retroperitoneale Fibrose - Periaortitis - Pseudotumor - Autoimmunpankreatitis

Keywords

IgG4 - retroperitoneal fibrosis - periaortitis - pseudotumor - autoimmunepancreatitis

Publikationsverlauf

Eingereicht: 14. Februar 2025

Angenommen: 22. April 2025

Artikel online veröffentlicht:
04. Juni 2025

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