Autoimmunpankreatitis

Zusammenfassung

Die Autoimmunpankreatitis ist eine seltene Form der chronischen Pankreatitis, die sich durch ein lymphoplasmazelluläres Infiltrat mit storiformer Fibrose auszeichnet und häufig durch einen schmerzlosen Ikterus und diskrete Oberbauchbeschwerden auffällig wird. Es werden zwei klinische Typen unterschieden, die sich hinsichtlich Histologie, klinischem Bild und Prognose unterscheiden. Die AIP vom Typ 1 ist die pankreatische Manifestation des IgG4-assoziierten Syndroms und geht mit der Beteiligung weiterer Organsysteme einher. Ein Drittel der Typ 1 Patienten ist nur histologisch oder durch einen erfolgreichen Therapieversuch sicher zu diagnostizieren. Typ 2 ist eine IgG4-negative Form mit dem histologischen Bild einer idiopathischen gangzentrierten Pankreatitis (Idiopathic Duct Centric Pancreatitis, IDCP) und in erhöhtem Maße mit chronisch entzündlichen Darmerkrankungen assoziiert. Die sichere Diagnose gelingt nur mittels Biopsie. Beide Formen sprechen in der Regel gut auf Steroide an, beim Typ 1 erleiden jedoch bis zu 50 % der Patienten ein Rezidiv. Die größte Herausforderung und gleichzeitig wichtigste Differentialdiagnose ist die Unterscheidung vom Pankreaskarzinom, da die AIP häufig mit Herdbefunden, Lymphadenopathie und Gangobstruktion einhergeht. Der folgende Artikel fast die neusten Erkenntnisse zum Auftreten, klinischer Symptomatik, diagnostischer Strategie, Therapie und Differentialdiagnose dieser noch recht unbekannten Erkrankung zusammen.

Abstract

Autoimmune pancreatitis is a relatively rare form of chronic pancreatitis which is characterized by a lymphoplasmatic infiltrate with a storiform fibrosis and often goes along with painless jaundice and discrete discomfort of the upper abdomen. Clinically we distinguish between two subtypes, which differ in terms of their histology, clinical picture and prognosis. Type 1 autoimmune pancreatitis is the pancreatic manifestation of the IgG4-associated syndrome which also involves other organs. About one third of the patients can only be diagnosed after either histological prove or a successful steroid trail. Type 2 is IgG4-negative with the histological picture of an idiopathic duct centric pancreatitis and is to higher degree associated with inflammatory bowel disease. A definitive diagnosis can only be made using biopsy. Usually both forms show response to steroid treatment, but in type 1 up to 50 % of the patients might develop a relapse. The biggest challenge and most important differential diagnosis remains the discrimination of AIP from pancreatic cancer, because also AIP can cause mass of the pancreatic head, lymphadenopathy and ductal obstruction. This article summarizes recent advances on epidemiology, clinical presentation, diagnostic strategy, therapy and differential diagnosis in this relatively unknown disease.

Schlüsselwörter

IgG4 - Autoimmunpankreatitis Typ 1 - Autoimmunpankreatitis Typ 2 - HiSORT-Kriterien - Pankreaskarzinom

Keywords

IgG4 - autoimmune pancreatitis type 1 - autoimmune pancreatitis type 2 - HiSORT criteria - pancreatic cancer

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