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Aktuelle Rheumatologie 2012; 37(05): 299-305
DOI: 10.1055/s-0032-1321833
Übersichtsarbeit
© Georg Thieme Verlag KG Stuttgart · New York

Immunglobulin G4 (IgG4)-assoziierte hepatobiliäre Erkrankungen

Immunglobulin G4 (IgG4)-Associated Hepatobiliary Diseases
T. Müller
1   Medizinische Klinik m. S. Hepatologie und Gastroenterologie, Charité – Universitätsmedizin Berlin, Campus Virchow Klinikum Berlin
,
T. Berg
2   Sektion Hepatologie, Klinik und Poliklinik für Gastroenterologie und Rheumatologie, Universitätsklinikum Leipzig, Leipzig
› Author Affiliations
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Publication History

Publication Date:
05 October 2012 (online)

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Zusammenfassung

Die IgG4-assoziierte Cholangitis (IAC) stellt neben der IgG4-assoziierten Hepatopathie die wichtigste hepatobiliäre Manifestation aus dem relativ neuen Formenkreis der IgG4-assoziierten sklerosierenden Autoimmunerkrankungen („IgG4-related sclerosing disease“ ISD) dar. Die autoimmune Pankreatitis (AIP) ist die am besten charakterisierte pankreatische Manifestation der ISD. Die IAC zeichnet sich durch zumeist multifokal auftretende inflammatorische und fibrotische Stenosierungen der intra- und extra-hepatischen Gallengänge aus. Im Gegensatz zu der häufig schwierig zu differenzierenden primär sklerosierenden Cholangitis (PSC) weisen die Strikturen der IAC eine charakteristische Infiltration IgG4-exprimierender (IgG4[+]) Plasmazellen auf. Zudem finden sich häufig – aber nicht immer – erhöhte IgG4-Serumspiegel bei diesen Patienten. Im Gegensatz zur PSC sprechen die entzündlichen Gallengangsstenosen einer IAC in der Regel gut auf Steroide an. Offenbar existieren auch Überlappungsformen mit der PSC und der autoimmunen Hepatitis (AIH). Patienten mit PSC sowie AIH und positivem IgG4 Status zeigen signifikante Unterschiede im natürlichen Langzeit-Verlauf sowie im Therapie-Ansprechen im Vergleich zu Patienten mit „klassischer“ PSC bzw. AIH ohne erhöhte IgG4 Serumspiegel. Es ist aber zurzeit unklar, ob es sich hierbei um eigenständige Krankheitsentitäten oder um Subgruppen der „klassischen“ PSC bzw. AIH handelt. Die Abgrenzung der IAC zum Cholangiokarzinom (CCA) kann ebenfalls äußerst problematisch sein. Die Einbeziehung der IAC in die Differenzialdiagnose jeder unklaren Gallengangsstenose ist jedoch aufgrund des unterschiedlichen therapeutischen Managements von entscheidender Bedeutung.

Abstract

IgG4-associated cholangitis (IAC) and IgG4-associated hepatopathies constitute the most important hepatobiliary manifestations of the recently described new chronic inflammatory disease entity “IgG4-related sclerosing disease” (ISD). ISD is a systemic steroid-responsive fibro-inflammatory disorder characterised by dense infiltration of affected organs with IgG4-positive lymphoplasmacytic infiltrates and is frequently accompanied by elevated IgG4 serum levels. Autoimmune pancreatitis (AIP) represents the best characterised pancreatic manifestation of ISD and in most patients AIP is indicative of this multisystem disease. IAC is often difficult to distinguish from “classical PSC. In contrast to PSC”, IAC shows features of an autoimmune cholangitis with extra- and intrahepatic biliary strictures which promptly respond to steroid treatment. The differentiation of IAC from malignant disorders of the biliary tract (i. e., cholangiocarcinoma of the intra- and extrahepatic bile ducts or pancreatic head carcinoma) can be very challenging in the daily practice and is of great importance for subsequent treatment decisions. Finally, PSC patients with elevated IgG4 serum levels [IgG4(+)PSC] have also been described and may constitute a special steroid-sensitive subpopulation of PSC patients with a worse prognosis than “classical” PSC patients with normal IgG4 serum levels. Similary, patients with classical autoimmune pancreatitis (AIH) without elevated IgG4 serum levels might need to be distinguished from AIH patients with elevated IgG4 serum levels [IgG4(+)AIH]. However, it remains to be determined whether IAC, PSC and IgG4(+)PSC, respectively, AIH and IgG4(+)AIH represent overlapping variants of the same disease spectrum or distinct disease entities.

Schlüsselwörter

IgG4-assoziierte sklerosierende Autoimmunerkrankung - IgG4-assoziierte Cholangiopathie - primär sklerosierende Cholangitis - Cholangiokarzinom - IgG4-assoziierte Hepatopathie

Key words

IgG4-related sclerosing disease - IgG4-associated cholangiopathy - primary sclerosing cholangitis - cholangiocarcinoma - IgG4-associated hepatopathy
 

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