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Autoimmunpankreatitis

Diagnostik und Therapie

Autoimmune pancreatitis

Diagnosis and therapy

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Der Gastroenterologe Aims and scope

Zusammenfassung

Die Autoimmunpankreatitis (AIP) ist eine seit gut 15 Jahren bekannte autoinflammatorische Erkrankung des Pankreas. Die Ätiologie ist unbekannt; ca. 5% aller chronischen Pankreatitiden sind autoimmun. Entsprechend den jüngsten internationalen Konsensuskonferenzen werden 2 Typen unterschieden: Typ 1 als lymphoplasmazytäre sklerosierende Pankreatitis mit IgG4-positiven Zellen und Typ 2 als idiopathische duktozentrische chronische Pankreatitis mit granulozytären epithelialen Läsionen und Eosinophilen. Dieser histologischen Klassifikation können unterschiedliche klinische Präsentationen zugeordnet werden: Der AIP-Typ 1 ist durch Serumerhöhungen von IgG und IgG4, häufigerem Relaps und häufigerer Beteiligung anderer Organe gekennzeichnet. Der AIP-Typ 2 hat dagegen ein geringeres Relapsrisiko, ist nicht IgG/IgG4-positiv, weist seltener eine Beteiligung anderer Organe auf, besitzt aber eine höhere Assoziation mit Morbus Crohn. Die Diagnosestellung folgt einem Algorithmus, basiert aber vor allem auf der Bildgebung (CT, MRT), die häufig pathognomonisch ist. Die Serologie ist unzuverlässig. Bei AIP-Verdacht nimmt die endosonographisch gesteuerte Feinnadelpunktion eine Schlüsselrolle ein. Sobald die Diagnose gesichert ist, kann mit Steroiden behandelt werden. Die allermeisten Patienten sind nach einem initialen Kortisonzyklus geheilt und bedürfen keiner weiteren Therapie. Relapse können nochmals mit Kortikoiden oder im Falle der Resistenz mit anderen gängigen Immunsuppressiva behandelt werden.

Abstract

Autoimmune pancreatitis (AIP) has been established for more than 15 years as a disease entity of chronic pancreatitis. Although the autoimmune etiology is well established AIP is still an enigmatic disease. Morphological hallmarks of the disease are narrowing of the pancreatic duct system and frequently even narrowing of the bile duct by periductal lymphoplasmocytic inflammation. In cases of more localized inflammation obstructive jaundice due to a mass-forming lesion in the pancreatic head may mimic pancreatic ductal adenocarcinoma. Therefore, even in tertiary referral center, AIP patients underwent pancreatic surgery for suspected pancreatic cancer due to misclassification. Histopathologically, the disease can be differentiated into lymphoplasmacytic sclerosing pancreatitis (LPSP) with IgG4-positive cells or idiopathic ductocentral chronic pancreatitis (IDCP) with granulocyte epithelial lesions and eosinophils. Recently, LPSP has been coined AIP type 1 and IDCP as AIP type 2 following a recent consensus conference. Serologically, patients may present with elevated serum IgG and IgG4 levels. Additionally, autoantibodies have been described targeting ductal and acinar antigens. Association with other autoimmune manifestations in a wide range of organs is relatively frequent. In most cases, autoimmune pancreatitis responds to steroid treatment very quickly, which is of specific importance when considering the differential diagnosis of pancreatic cancer.

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  1. Gastrocentrum, Karolinska Institutet, CLINTEC, K53, 141 86, Stockholm, Schweden

    J.-M. Löhr & S.L. Haas

  2. Institut für Pathologie, Karolinska University Hospital, Stockholm, Schweden

    C. Verbeke

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Diese Arbeit ist Jörg Emmrich (1955–2011) gewidmet.

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Löhr, JM., Verbeke, C. & Haas, S. Autoimmunpankreatitis. Gastroenterologe 7, 493–506 (2012). https://doi.org/10.1007/s11377-011-0622-1

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