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Erkrankungen des Bindegewebes bei IgG4-assoziierten Autoimmunerkrankungen

Diseases of connective tissue in IgG4-associated autoimmune diseases

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Zusammenfassung

IgG4-assoziierte Erkrankungen des Bindegewebes beruhen auf einer allergenvermittelten Autoimmunreaktion und können das Retroperitoneum, das Mesenterium, das Mediastinum sowie seltener andere Lokalisationen (Hals, Orbita und periartikuläres Bindgewebe) betreffen. Die inflammatorische Fibrosierung mit zunächst lymphozyten- und plasmazellreichem Infiltrat und zellarmer storiformer Fibrose im späteren Stadium kann durch die Stenosierung oder den Verschluss von Gefäßen, Ureteren oder Darmschlingen schwere Komplikationen auslösen. Mithilfe von CT und MRT können die Differenzialdiagnose eingegrenzt, die Ausprägung dargestellt und Komplikationen aufgedeckt werden.

Abstract

The IgG4-associated diseases of connective tissue are caused by allergen-mediated autoimmune processes. The retroperitoneum, mesentery and mediastinum as well as other less common localizations, such as the neck, orbit and periarticular connective tissue can be affected. Inflammatory fibrosis is initially dominated by a lymphocytic and plasma cell-rich infiltrate and later by cell-poor storiform fibrosis. Stenosis and occlusion of vessels, ureters and intestines can cause severe complications. Computed tomography (CT) and magnetic resonance imaging (MRI) can be implemented in the diagnostic algorithm to narrow the differential diagnosis, delineate the distribution of fibrosis and reveal complications.

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  1. Klinik für Diagnostische und Interventionelle Radiologie, Justus-Liebig-Universität Giessen, Klinikstrasse 33, 35392, Giessen, Deutschland

    G. A. Krombach

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Krombach, G.A. Erkrankungen des Bindegewebes bei IgG4-assoziierten Autoimmunerkrankungen. Radiologe 56, 1061–1068 (2016). https://doi.org/10.1007/s00117-016-0183-x

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  • DOI: https://doi.org/10.1007/s00117-016-0183-x

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