IgG4-related disease (IgG4-RD) – auch eine Erkrankung des Kindesalters

Zusammenfassung

IgG4-related disease (IgG4-RD) ist eine neu beschriebene entzündlich fibrosierende Erkrankung unklarer Ätiologie, die eine Vielzahl von Organen befallen kann. Initial kommt es zur Organvergrößerung im Rahmen einer Entzündungsreaktion, die im Verlauf zu fibrotischen Veränderungen mit Funktionseinschränkung führt. Je nach Organbefall kann die Klinik vielgestaltig sein. In der laborchemischen Analyse können Entzündungsparameter, insbesondere CRP und BSG, erhöht sein. Serumkonzentrationen von IgG4 sind oft, aber nicht immer pathologisch hoch. Die Diagnose wird durch eine histologische und immunhistochemische Untersuchung von Biopsaten der betroffenen Organe gesichert. Diagnostisch wichtig sind dabei dichte lymphoplasmazelluläre Infiltrate, eine obliterative Phlebitis, eine geschichtete/gewirbelte Fibrose, eine Gewebs-Eosinophilie und eine erhöhte Zahl von IgG4-positiven Plasmazellen im Gewebe. Therapiestudien zum IgG4-RD existieren nicht. Im entzündlichen Stadium wird je nach Symptomatik eine Therapie mit Glukokortikoiden empfohlen. Bei schweren Verläufen werden Methotrexat, Mycophenolat-Mofetil und Azathioprin eingesetzt. Als Reservemedikament steht Rituximab zur Verfügung.

Summary

IgG4-related disease (IgG4-RD) is a recently described fibro-inflammatory disease of unknown etiology that may affect various organs. The initial clinical finding is organ enlargement caused by inflammation, which may lead to fibrotic changes and organ dysfunction. Clinical symptoms are variable depending on the affected organs. The laboratory analysis may show an increase in inflammatory parameters (CRP, ESR). Serum concentrations of IgG4 are often, but not always, elevated. The diagnosis is based on histopathological and immunohistochemical changes in affected organs. The following pathological signs are required for diagnosis: dense lymphoplasmacytic infiltrates, obliterative phlebitis, storiform fibrosis, tissue eosinophilia, and an elevated number of IgG4-positive plasma cells in the tissue. There are no therapeutic studies on IgG4-RD. During the inflammatory stage, and depending on the symptoms and the extent of organ involvement, glucocorticoids are recommended. In severe cases, methotrexate, mycophenolate mofetil and azathioprine may be used. Rituximab is a second line drug.

Schlüsselwörter

IgG4-related disease (IgG4-RD) - Fibroinflammation - immunsuppressive Therapie

Keywords

IgG4-related disease (IgG4-RD) - fibro-inflammation - immunosuppressive therapy

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