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Autoimmune Pankreatitis

Autoimmune pancreatitis

Zusammenfassung

Die autoimmune Pankreatitis wird als eigenständige Form der Pankreatitis angesehen. Sie ist eine rätselhafte Erkrankung, die eine autoimmune Ätiologie mit den bekannten Ursachen der Pankreatitis verbindet. Morphologische Kennzeichen dieser Erkrankung sind Verengungen des pankreatischen Gangsystems und des Gallengangs durch eine periduktale lymphoplasmazelluläre Entzündung. Dies führt in vielen Fällen zum Ikterus aufgrund einer raumfordernden Läsion im Pankreaskopf, die einem duktalen Adenokarzinom des Pankreas ähnelt. Entsprechend werden viele Patienten einer Operation unterzogen. Histopathologisch kann die Erkrankung anhand IgG4-positiver Plasmazellen diagnostiziert werden. Serologisch zeigen viele Patienten erhöhte Serum-IgG- und -IgG4-Spiegel. Verschiedene Antikörper werden beschrieben. Die Assoziation mit anderen Autoimmunerkrankungen in diversen Organen ist häufig. Die autoimmune Pankreatitis spricht auf eine Steroidtherapie an, dies kann zur Abgrenzung der klinischen Differenzialdiagnose des Pankreaskarzinoms dienen. Die positive Diagnose der Autoimmunpankreatitis ist auch deshalb von Bedeutung, weil eine Cholangitis, insbesondere wenn Gallengänge betroffen sind, vor oder nach einer Operation als Komplikation auftreten kann.

Abstract

Autoimmune pancreatitis has been established as a special entity of pancreatitis. It is an enigmatic disease since it is adding an autoimmune etiology to the existing causes of pancreatitis. Morphological hallmarks of the disease are narrowing of the pancreatic duct system and the bile duct by periductal lymphoplasmocytic inflammation. This results in many cases in obstructive jaundice due to a mass-forming lesion in the pancreatic head mimicking pancreatic ductal adenocarcinoma. Therefore, patients will frequently undergo surgery. Histopathologically, the disease can be diagnosed by IgG4-positive plasma cells. Serologically, patients may present with elevated serum IgG and IgG4 levels. Other autoantibodies are also described. Association with other autoimmune manifestations in a wide range of organs is frequent. Autoimmune pancreatitis will respond to steroid treatment, which is of specific importance because pancreatic cancer is one of its clinical differential diagnoses. It is important to positively diagnose autoimmune pancreatitis, especially if the bile ducts are affected, since cholangitis may be or become a prominent problem before or after surgery.

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Schneider, A., Löhr, J. Autoimmune Pankreatitis. Internist 50, 318–330 (2009). https://doi.org/10.1007/s00108-008-2262-1

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Schlüsselwörter

  • Autoimmune Pankreatitis
  • Chronische Pankreatitis
  • Autoantikörper
  • Autoimmunerkrankung

Keywords

  • Autoimmune pancreatitis
  • Chronic pancreatitis
  • Autoantibodies
  • Autoimmune disease