IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma

Abstract

IgG4-related disease has gained increased attention worldwide. While the initial focus was on autoimmune pancreatitis which was first described in Asian populations and turned out to be of relevance in Western populations too, the scope has recently broadened towards a notion of a multi-systemic disease with very diverse manifestations such as autoimmune pancreatitis, IgG4-related sclerosing cholangitis (IgG4-SC), retroperitoneal fibrosis and tubulointerstitial nephritis. IgG4-SC (also known as IgG4-associated cholangitis, IAC) represents a rare but clinically challenging differential diagnosis in patients with obstructive jaundice and proximal extra- or intrahepatic biliary strictures which can be mistaken for cholangiocarcinoma (CC). We present the case of a 79-year-old male patient who presented with obstructive jaundice and biliary strictures at the hepatic duct bifurcation without any evidence for autoimmune pancreatitis and without elevation of serum IgG4-concentrations who underwent hemihepatectomy for suspected CC. However, on histological examination of the resection specimen CC could not be confirmed. It was only after several episodes of obstructive jaundice had reoccurred that the diagnosis of IgG4-SC could be established by reexamination of the surgical specimen which showed extensive infiltration with IgG4-positive plasma cells. Appropriate medical treatment with steroids and azathioprine led to complete remission of the disease. Early recognition of IgG4-SC can save patients from potential harmful and unnecessary surgical interventions. Here we describe the clinical features of this rare case of IgG4-SC with extensive liver tissue infiltration with IgG4-positive cells but without elevated serum IgG4 concentration or evidence of autoimmune pancreatitis. We describe diagnostic criteria for IgG4-SC and review recent insights in pathophysiology and treatment options.

Zusammenfassung

IgG4-assoziierte Erkrankungen haben weltweit zuletzt zunehmend Beachtung gefunden. Während zunächst die autoimmune Pankreatitis im Fokus stand, die erstmalig in asiatischen Patienten beschrieben worden war und deren Relevanz im Verlauf auch in westlichen Populationen erkannt wurde, wurden IgG4-assoziierte Erkrankungen in den vergangenen Jahren zunehmend im Sinne einer Multisystemerkrankung mit äußerst unterschiedlichen Manifestationen wie der autoimmunen Pankreatitis, der IgG4-assoziierten sklerosierenden Cholangitis (IgG4-SC), der Retroperitonealfibrose oder der tubulointerstitiellen Nephritis wahrgenommen. Die IgG4-SC – auch als IgG4-assoziierte Cholangitis (IAC) bezeichnet – stellt eine seltene, aber klinisch herausfordernde Differenzial­diagnose zum cholangiozellulären Karzinom bei Patienten mit Ikterus und proximalen extra- oder intrahepatischen Gallenwegsstenosen dar. Wir beschreiben den Fall eines 79-jährigen männlichen Patienten, der sich mit Ikterus und suspekten Gallenwegsstenosen im Bereich der Gallengangsbifurkation ohne Hinweise auf eine autoimmune Pankreatitis und ohne erhöhte IgG4-Serumkonzentration vorstellte. Unter der Verdachtsdiagnose eines cholangiozellulären Karzinoms erfolgte eine Hemihepatektomie. In der histopathogischen Aufarbeitung des Operationspräparats fand sich allerdings kein Hinweis auf einen malignen Prozess. Erst nachdem es zu mehreren weiteren ikterischen Episoden gekommen war, konnte im Rahmen einer Nachuntersuchung des Resektats eine ausgedehnte Infiltration mit IgG4-positiven Plasmazellen nachgewiesen werden. Durch eine entsprechende medikamentöse Therapie mit Kortikoiden und Azathioprin konnte eine rasche und vollständige Remission der Erkrankung erreicht werden. Die frühzeitige Diagnose einer IgG4-SC kann eine unnötige und potenziell risikobehaftete chirurgische Intervention verhindern. Anhand dieses sehr seltenen Falles mit ausgedehnter IgG4-positiver Lebergewebeinfiltration, aber ohne erhöhte IgG4-Serumkonzentration oder Vorliegen einer autoimmunen Pankreatitis, werden die klinischen Merkmale sowie diagnostische Kriterien der IgG4-SC vorgestellt. Wir diskutieren neue Erkenntnisse in die Pathophysiologie sowie die Behandlungsmodalitäten dieser Erkrankung.

Key words

liver - cholangitis - cholangiocarcinoma - IgG4 - autoimmune pancreatitis - retroperitoneal fibrosis

Schlüsselwörter

Leber - Cholangitis - cholangiozelluläres Karzinom - IgG4 - autoimmune Pankreatitis - Retroperitonealfibrose

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