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Die autoimmune Pankreatitis — eine chirurgische Krankheit?

Autoimmune pancreatitis — a surgical disease?

Zusammenfassung

Der Begriff der autoimmunen Pankreatitis (AIP) beschreibt eine nichtalkoholinduzierte, chronische lymphoplasmozytäre Pankreatitis. Periduktale lymphoplasmozytäre Infiltrate mit einem Übergewicht an CD4-positiven T-Zellen sind häufig von einer Fibrose und Azinuszellatrophie begleitet, was zu einer Stenosierung des Ductus wirsungianus und des distalen Ductus choledochus führen kann. In der bildgebenden Diagnostik spiegelt sich dies oftmals in einer unregelmäßigen Stenosierung des Pankreashauptgangs und einem entzündlich bedingten Volumenplus vor allem des Pankreaskopfes wider, was nur schwer von einem Malignom zu differenzieren ist. Klinisch finden sich uncharakteristische Oberbauchschmerzen, gelegentlich ein Verschlussikterus, rezidivierende Pankreatitisschübe oder ein neu aufgetretener Diabetes mellitus. Die AIP kann zudem mit autoimmunen Erkrankungen wie dem Sjögren-Syndrom, der primär sklerosierenden Cholangitis, chronisch entzündlichen Darmerkrankungen und der retroperitonealen Fibrose assoziiert sein. Serologisch können häufig Autoantikörper, eine Hypergammaglobulinämie und vor allem erhöhte Werte für IgG4 nachgewiesen werden. Charakteristisch scheint auch die Wirksamkeit einer Steroidtherapie zu sein, worunter eine Besserung der klinischen Symptome und eine Rückbildung der Pankreas- und Gallengangsstenosen beobachtet wurden. Dadurch unterscheidet sich die AIP grundlegend von anderen Pankreatitisformen und Pankreasneoplasien. In diesem Beitrag fassen wir den aktuellen Wissensstand über die AIP zusammen und berichten über insgesamt 17 Patienten mit AIP, die im Zeitraum von 2003–2004 in unserer Klinik operiert worden sind.

Abstract

The term autoimmune pancreatitis (AIP) describes a nonalcoholic, chronic lymphoplasmocytic pancreatitis. The lymphoplasmocytic infiltration is characterized by periductal localization of predominantly CD4-positive T cells, fibrosis, and acinar atrophy, frequently resulting in stenosis of the main pancreatic and distal common bile ducts. Imaging studies often reveal a diffuse narrowing of the pancreatic main duct and swelling of the pancreatic head wrongly suggesting the presence of a malignant tumor. Clinical signs include mild abdominal pain, jaundice, recurrent episodes of acute pancreatitis, and even new-onset diabetes mellitus. Additionally, AIP can be associated with other autoimmune diseases such as Sjögren’s syndrome, primary sclerosing cholangitis, chronic inflammatory bowel diseases, and retroperitoneal fibrosis. Serological markers include autoantibodies and increased levels of gamma globulin and especially IgG4. Steroids seem to be effective in improving clinical symptoms as well as in the resolution of pancreatic and bile duct narrowing. This distinguishes AIP from other forms of pancreatitis and from pancreatic neoplasms. Further studies of the underlying pathophysiologic mechanisms, prognosis, and new diagnostic tools are needed to provide adequate and effective treatment in the future. In this article, we summarize the current knowledge about AIP and present 17 cases that underwent surgical resection at our institution from 2003 to 2004.

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Abbreviations

ACA-II:

Anticarboanhydrase-II-Antikörper

AIP:

autoimmune Pankreatitis

ALF:

Antilaktoferrinantikörper

ANA:

antinukleärer Antikörper

ANCA:

antineutrophile zytoplasmatische Antikörper

AMA:

antimitochondriale Antikörper

CED:

chronisch entzündliche Darmerkrankung

DEPKR:

duodenumerhaltende Pankreaskopfresektion

GAD:

Glutamatdecarboxylase

Ig:

Immunglobulin

PBC:

primär biliäre Zirrhose

PSC:

primär sklerosierende Cholangitis

PPDP:

pyloruserhaltende partielle Duodenopankreatektomie

RF:

Rheumafaktor

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Kleeff, J., Welsch, T., Esposito, I. et al. Die autoimmune Pankreatitis — eine chirurgische Krankheit?. Chirurg 77, 154–165 (2006). https://doi.org/10.1007/s00104-005-1084-0

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Schlüsselwörter

  • Autoimmune Pankreatitis
  • Pankreastumor
  • IgG4
  • Steroide

Keywords

  • Autoimmune pancreatitis
  • Pancreatic tumor
  • IgG4
  • Steroids