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Die IgG4-assoziierte Erkrankung

Mikroskopische Diagnose und Differenzialdiagnose

IgG4-related disease

Microscopic diagnosis and differential diagnosis

  • Schwerpunkt: Seltene Erkrankungen
  • Published:
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Zusammenfassung

Bei dem als „IgG4-assoziierte Erkrankung“ bezeichneten Krankheitsbild handelt es sich um einen potenziell systemischen, chronisch entzündlichen und fibrosierenden Prozess. Unabhängig von der Organmanifestation sind die histologischen Hauptmerkmale ein lymphoplasmazelluläres Infiltrat, eine storiforme Fibrose und eine obliterative Phlebitis. Diese Hauptmerkmale werden jedoch in bestimmten Organen unterschiedlich gewichtet. Wenn mehrere dieser Merkmale vorliegen bei Abwesenheit von Veränderungen, die gegen die IgG4-RD sprechen, kann die Diagnose immunhistochemisch durch die Erfassung einer erhöhten Anzahl IgG4-positiver Plasmazellen und eines erhöhten IgG4-IgG-Quotienten und serologisch durch erhöhte IgG4-Werte weiter gestützt werden. Eine Gewebebiopsie stellt einen der Grundpfeiler in der Diagnostik dar, da die IgG4-assoziierte Erkrankung klinisch und in der Bildgebung oftmals einen Tumorprozess imitiert.

Abstract

IgG4-related disease (IgG4-RD) is a potentially systemic inflammatory and fibrosing disease. Independent of the affected organ, the main histological features are a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. These features are weighted slightly different, dependent on the respective organ. If several of these features are present and changes inconsistent with IgG4-RD are absent, the diagnosis is further supported by the immunohistochemical demonstration of increased IgG4-positive plasma cells and an elevated IgG4-IgG ratio, as well as serologically by elevated IgG4 levels. A tissue biopsy is often mandatory for the diagnosis, as IgG4-RD clinically and radiologically can imitate a malignant tumor.

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Authors and Affiliations

  1. Abteilung für Pathologie, Odense Universitätshospital, J. B. Winsløws Vej 15, 5000, Odense, Dänemark

    S. Detlefsen Ph.D.

  2. Odense Pancreas Center (OPAC), Odense Universitätshospital, Odense, Dänemark

    S. Detlefsen Ph.D.

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Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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C. Röcken, Kiel

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Detlefsen, S. Die IgG4-assoziierte Erkrankung. Pathologe 40, 619–626 (2019). https://doi.org/10.1007/s00292-019-00685-8

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