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IgG4-assoziierte Autoimmunerkrankungen

IgG4-assoziierte Pankreatitis und IgG4-assoziierte Cholangitis

IgG4-associated autoimmune diseases

IgG4-associated pancreatitis and IgG4-associated cholangitis

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Die Gastroenterologie Aims and scope

Zusammenfassung

Immunglobulin-G4(IgG4)-assoziierte Erkrankungen („IgG4-related diseases“, IgG4-RD) sind selten auftretende Autoimmunerkrankungen, die als Multisystemerkrankungen auch mehrere Organe gleichzeitig betreffen können. Je nach betroffenen Organen kann die Symptomatik sehr unterschiedlich sein. Die Diagnose einer IgG4-RD richtet sich nach dem Vorhandensein einer typischen Organschwellung und/oder Fibrose des betroffenen Organs, entsprechender Klinik, serologischen Veränderungen mit erhöhter IgG4-Konzentration im Serum (≥ 135 mg/dl) und einer pathognomonischen Histopathologie mit lymphozellulärem Infiltrat mit hohem Anteil an IgG4-positiven Plasmazellen (> 10/HPF [„high-power field“]; IgG4/IgG-positive Plasmazellen ≥ 40 %). Für die Diagnostik wurden für IgG4-RD im Allgemeinen wie auch für dezidierte Organmanifestationen wie die IgG4-assoziierte Autoimmunpankreatitis (IgG4-AIP) und die IgG4-assoziierte Cholangitis (IAC) organspezifische Kriterien bzw. Scores entwickelt. Als Goldstandard der Diagnostik sollte eine histopathologische Sicherung angestrebt werden. Nach Diagnose einer IgG4-RD sollte eine immunsuppressive Therapie durchgeführt werden. Als Standard sollte eine Kortikosteroidherapie als Induktionstherapie und niedrig dosiert zur Rezidivprophylaxe als Erhaltungstherapie für 1 Jahr durchgeführt werden. Rezidivierende Schübe einer IgG4-RD können zur Organfibrose mit konsekutivem Funktionsverlust des betroffenen Organs führen. Besteht ein streoidrefraktärer Verlauf oder eine Kontraindikation gegen eine Therapie mit Steroiden kann eine Therapie mit Azathioprin oder Rituximab erwogen werden.

Abstract

Immunoglobulin G4-related diseases (IgG4-RD) are rare autoimmune diseases, which as multisystemic diseases can also affect several organs simultaneously. The symptoms can be very different depending on the organ affected. The diagnosis of IgG4-RD is based on the presence of typical organ swelling and/or fibrosis of the affected organ, corresponding clinical symptoms, serological alterations with elevated serum concentrations of IgG4 (≥ 135 mg/dl) and a pathognomonic histopathology with predominant lymphocellular infiltration and a high proportion of IgG4 positive plasma cells (> 10 plasma cells/high-power field, HPF; IgG4/IgG positive plasma cells ≥ 40%). For the diagnostics of IgG4-RD in general as well as for specific organ manifestations, such as IgG4-associated autoimmune pancreatitis (IgG4-AIP) and IgG4-associated cholangitis (IAC) organ-specific criteria and scores have been developed. As the diagnostic gold standard histopathological confirmation should be strived for. After diagnosis of an IgG4-RD immunosuppressive treatment should be carried out. The standard treatment consists of corticosteroid treatment as induction phase and low-dosed for recurrence prophylaxis as maintenance treatment for 1 year. Recurrent exacerbations of IgG4-RD can lead to organ fibrosis with subsequent loss of function of the affected organ. In cases of contraindications to steroid treatment or a steroid-refractory course, treatment with azathioprine or rituximab can be considered.

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Authors and Affiliations

  1. Klinik und Poliklinik für Innere Medizin I, Universitätsklinikum Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Deutschland

    Patricia Mester, Bernhard Michels, Alexander Mehrl, Martina Müller-Schilling &  Arne Kandulski

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  1. Patricia Mester
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Correspondence to Arne Kandulski.

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Interessenkonflikt

A. Kandulski: Forschung/klinische Studien: Roche Pharma AG; Eisai GmbH; Abbvie Deutschland; Bristol Myers Squibb. Referent/Beratertätigkeit: Roche Pharma AG; Eisai GmbH; Abbvie Deutschland, Janssen-Cilag; Boston Scientific Corp. Micro-Tech Europe, Bayer Vital GmbH. P. Mester, B. Michels, A. Mehrl und M. Müller-Schilling geben an, dass kein Interessenkonflikt besteht.

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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Martina Müller-Schilling, Regensburg

Stefan Zeuzem, Frankfurt am Main

Die Autoren Patricia Mester und Bernhard Michels haben zu gleichen Teilen zum Manuskript beigetragen.

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Mester, P., Michels, B., Mehrl, A. et al. IgG4-assoziierte Autoimmunerkrankungen. Gastroenterologie 17, 325–334 (2022). https://doi.org/10.1007/s11377-022-00637-4

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