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Autoimmunpankreatitis – „the new kid on the block“

Autoimmune pancreatitis—the new kid on the block

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Der Gastroenterologe Aims and scope

Zusammenfassung

Die Autoimmunpankreatitis (AIP) ist ein seltenes Krankheitsbild, das sich durch eine diffuse oder fokale Entzündung des Pankreas, ein lymphoplasmazelluläres Infiltrat mit Fibrosebildung und das Ansprechen auf Steroide auszeichnet. Die AIP wird in 3 Subtypen unterteilt: die lymphoplasmazelluläre sklerosierende Pankreatitis (LPSP; AIP Typ 1), die idiopathische duktozentrische Pankreatitis (IDCP; AIP Typ 2) und die nicht weiter spezifizierte Pankreatitis (NOS). Die AIP Typ 1 ist die pankreatische Manifestation des IgG4-assoziierten Syndroms, während Typ 2 eine IgG4-negative, auf das Pankreas beschränkte Erkrankung mit Assoziation zu chronisch-entzündlichen Darmerkrankungen darstellt. Epidemiologischen Studien zufolge liegt in Japan die Erkrankungshäufigkeit der AIP bei 0,82 pro 100. 000 Einwohner, in Europa wird eine AIP bei zirka 5–6 % aller chronischen Pankreatitiden diagnostiziert. Das durchschnittliche Erkrankungsalter liegt jenseits des 50. Lebensjahrs. Die Pathogenese der AIP ist bisher noch nicht vollständig geklärt. Angekommen wird eine multifaktorielle Genese bedingt durch genetische Prädisposition sowie den Einfluss von exogenen Faktoren. Patienten mit einer AIP weisen unspezifische Symptome auf, die denen eines Pankreaskarzinoms ähneln können. Die Diagnosestellung der AIP erfolgt nach den HISORt-Kriterien, einer Kombination von Kriterien, die die Histologie (H), Bildgebung (I), IgG4-Serologie (S), die Beteiligung anderer Organsysteme (O) und das Ansprechen auf eine Steroidtherapie (Rt) einschließen. Die Prognose der Erkrankung wird insgesamt jedoch als recht gut eingestuft: Bei über 90 % der Patienten kann mit Steroiden eine Remission erzielt werden.

Abstract

Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis which is characterized by diffuse or focal pancreatic enlargement, a lymphoplasmacytic infiltrate with a storiform fibrosis and a dramatic response to steroid treatment. AIP can be classified into three subtypes: lymphoplasmacytic sclerosing pancreatitis (LPSP; AIP type 1), idiopathic duct centric pancreatitis (IDCP; AIP type 2), and not otherwise specified (NOS). AIP type 1 is the pancreatic manifestation of IgG4-related disease which also involves other organs; AIP type 2 is a pancreas-specific, IgG4-negative disorder and is associated with inflammatory bowel disease. Epidemiological studies from Japan estimate an incidence of approximately 0.82 per 100,000 inhabitants; in Europe 5–6% of all patients with chronic pancreatitis are diagnosed with AIP. The pathomechanism of AIP remains enigmatic, but a mulitfactorial etiology with genetic predisposition and environmental influences has been proposed. Clinically, patients can present with jaundice, epigastric pain, weight loss and new-onset diabetes, thus, raising suspicion for pancreatic malignancy. A combination of the HISORt (Histology, Imaging, IgG4 Serology, other Organ involvement and Response to steroid therapy) criteria can be used to definitively diagnose AIP in most cases. The greatest challenge is to differentiate AIP from pancreatic cancer and cholangiocarcinoma, since it often presents with a pancreatic mass, lymphadenopathy and bile duct obstruction. The prognosis of AIP is usually favorable; in over 90% of cases remission can be induced by steroid treatment.

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Förderung

Diese Arbeit wurde durch die Deutsche Krebshilfe/Dr. Mildred-Scheel-Stiftung (109102), die Europäische Union (EU-FP-7: EPC-TM, PePPP Exzellenzinitiative MV ESF/14-BM-A55-0045/16) und die DFG (SFB 1321-A14; RTG 1947 BioX A3) unterstützt.

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Authors and Affiliations

  1. Medizinische Klinik und Poliklinik 2, Klinikum der Universität München, Marchinioninistr. 15, 81377, München, Deutschland

    E. Goni, G. Beyer, J. Peterhansl, C. Schulz &  J. Mayerle

  2. Klinik und Poliklinik für Radiologie, Klinikum der Universität München, München, Deutschland

    M. Seidensticker

Authors
  1. E. Goni
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  2. G. Beyer
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  3. J. Peterhansl
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  4. M. Seidensticker
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  5. C. Schulz
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  6. J. Mayerle
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Correspondence to J. Mayerle.

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Interessenkonflikt

E. Goni, G. Beyer, J. Peterhansl, M. Seidensticker, C. Schulz und J. Mayerle geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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M. Fried, Zürich

R.M. Schmid, München

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Goni, E., Beyer, G., Peterhansl, J. et al. Autoimmunpankreatitis – „the new kid on the block“. Gastroenterologe 13, 425–435 (2018). https://doi.org/10.1007/s11377-018-0299-9

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