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Histopathologie der IgG4-RD

Histopathology of IgG4-related disease

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Zusammenfassung

Im Jahr 2011 wurden auf einer internationalen Konsensuskonferenz multifokale chronisch fibrosierende Entzündungsprozesse, denen erhöhte Serum-IgG4-Werte und/oder eine Gewebeinfiltration mit IgG4-positiven Plasmazellen gemeinsam ist, zur Krankheitsentität der IgG4-assoziierten Erkrankung – auf Englisch „IgG4-related disease“ (IgG4-RD) – zusammengefasst. Da die IgG4-RD ausgezeichnet auf Kortison anspricht, in den betroffenen Organen, insbesondere im Pankreas, aber oft einen Tumor vortäuscht, ist eine bioptische Sicherung der Diagnose häufig notwendig. Der histologische Kriterienkatalog, wie er 2011 für die IgG4-RD definiert wurde, basiert auf folgenden Hauptmerkmalen: 1) ein dichtes lymphoplasmazelluläres Infiltrat, 2) eine storiforme Fibrose und 3) eine obliterative Phlebitis. Zur weiteren Diagnosesicherung dient der immunhistochemische Nachweis einer erhöhten Infiltration mit IgG4-positiven Plasmazellen sowie einer erhöhten IgG4/IgG-Ratio. Die morphologischen Kriterien, welche die IgG4-RD charakterisieren, werden in den meisten Fällen in der Biopsie erfasst und können in Zusammenschau mit den klinischen, serologischen (erhöhter Serum-IgG4-Wert) und radiologischen Befunden die Diagnose sichern.

Abstract

At an international consensus conference in 2011, multifocal chronic fibrosing inflammatory processes, which are associated with elevated IgG4 serum levels and/or tissue infiltration with IgG4 positive plasma cells, were recognized as a distinct disease entity called IgG4-related disease (IgG4-RD). As IgG4-RD responds well to steroid treatment but imitates a tumor in many organs, particularly in the pancreas, a biopsy for confirmation of the diagnosis is often warranted. The histological criteria for IgG4-RD as defined in 2011 are based on the following main features: 1) dense lymphoplasmacytic infiltrate, 2) storiform fibrosis and 3) obliterative phlebitis. The diagnosis is further supported by immunohistochemical demonstration of an increased infiltration of IgG4-positive plasma cells and an elevated IgG4/IgG ratio. The morphological criteria of IgG4-RD are in most cases detectable in biopsies and can significantly contribute to the diagnosis of this disease, in concert with clinical, serological (elevated serum IgG4 level) and radiological features.

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Authors and Affiliations

  1. Abteilung für Pathologie, Odense Universitätshospital, Odense, Dänemark

    S. Detlefsen

  2. Institut für Pathologie, Konsultationszentrum für Pankreas und Endokrine Tumore, Technische Universität München, Ismaningerstr. 22, 81675, München, Deutschland

    G. Klöppel

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Correspondence to G. Klöppel.

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Interessenkonflikt

S. Detlefsen und G. Klöppel geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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P. Lamprecht, Lübeck

F. Moosig, Neumünster

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Detlefsen, S., Klöppel, G. Histopathologie der IgG4-RD. Z Rheumatol 75, 666–674 (2016). https://doi.org/10.1007/s00393-016-0130-2

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