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Autoimmune Pankreatitis – Behandlung und Problematik der Diagnosestellung

Autoimmune pancreatitis – treatment and pitfalls in diagnostics

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Zusammenfassung

Hintergrund

Die autoimmune Pankreatitis (AIP) wurde 1995 erstmals als eigene Identität definiert. Etwa 2 % aller chronischen Pankreatitiden (Prävalenz 36–41 pro 100.000) haben eine autoimmune Genese, und in 2,4 % der Pankreasresektionen findet sich eine AIP.

Fragestellung

Die Diagnose- und Behandlungsstrategien, insbesondere hinsichtlich der mitunter schwierigen Abgrenzung zum Pankreaskarzinom, werden dargestellt.

Methoden

Es wurde eine selektive Literaturrecherche mit PubMed bezüglich Pathogenese, Diagnostik und Therapie der AIP durchgeführt.

Ergebnisse

Die AIP ist durch rezidivierende Pankreatitiden mit Ikterus im Rahmen von Gallengangsstenosen, den histologischen Nachweis von Fibrose und eines lymphoplasmozytären oder granulozytären Infiltrats sowie das Ansprechen auf eine Steroidtherapie charakterisiert. Aus histologischer Sicht werden 2 Formen unterschieden: eine lymphoplasmozytische sklerosierende (Typ I) und eine idiopathische duktzentrische Pankreatitis(Typ II). Die IgG4-positive (IgG: Immunglobulin G) AIP Typ I zählt zu den IgG4-assoziierten Systemerkrankungen. Die Diagnose erfolgt analog den ICDC („international consensus diagnostic criteria“) oder den HISORt-Kriterien (HISORt: Akronym für „histology, imaging, serology, other organ involvement and response to therapy“) mittels Serologie, Bildgebung, Histologie und Therapieansprechen. Differenzialdiagnostisch schwierig kann die Abgrenzung zum Pankreaskarzinom sein. Goldstandard der Behandlung sind Kortikosteroide. Zur Reduktion der Steroiddosis ist eine Kombination mit Immunsuppressiva möglich. Bei steroidrefraktärer Erkrankung kann Rituximab zum Einsatz kommen. Indikation zur Behandlung sind Ikterus, eine systemische Manifestation sowie schwer beherrschbare Schmerzen.

Schlussfolgerungen

Autoimmune Pankreatitiden werden zunehmend diagnostiziert und können mit Kortikosteroiden, Immunsuppressiva und Rituximab erfolgreich konservativ behandelt werden. Trotz multimodaler Diagnosekriterien bleibt die Abgrenzung zum Pankreaskarzinom eine Herausforderung.

Abstract

Background

Autoimmune pancreatitis (AIP) was first classified as a defined disease entity in 1995. It accounts for approximately 2 % of cases of chronic pancreatitis (western world prevalence 36–41/100,000 inhabitants) and AIP is diagnosed in 2.4 % of pancreas resection specimens.

Objectives

Presentation of strategies for diagnosis and treatment with focus on differentiation of AIP and pancreatic carcinoma.

Methods

Selective literature research in PubMed regarding pathogenesis, diagnosis and treatment of AIP.

Results

Key characteristics of AIP are recurrent jaundice due to obstructed bile ducts, histological evidence of fibrosis, a lymphoplasmocytic or granulocytic infiltrate and the response to steroid therapy. There are two distinctive forms of AIP: type I or lymphoplasmocytic sclerosing pancreatitis and type II or idiopathic duct centric pancreatitis. The IgG4 positive AIP type I belongs to the group of IgG4-related systemic diseases. Diagnosis of AIP is established according to the international consensus diagnostic criteria (ICDC) or HISORt (mnemonic standing for histology, imaging, serology, other organ involvement and response to therapy) criteria. Differentiation from pancreatic adenocarcinoma can be challenging. The standard treatment consists of corticosteroids and in some cases azathioprine can be added. In refractory disease rituximab is a further option. Treatment is indicated in patients with jaundice, systemic manifestation or persistent pain.

Conclusion

Although AIP is increasingly being identified, the differentiation from pancreatic adenocarcinoma still remains difficult and in cases of a suspicion of neoplasia, resection should be favored. It can successfully be treated conservatively with steroids and rituximab.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. S. Rasch, V. Phillip, G. Weirich, I. Esposito, J. Gaa, R. M. Schmid und H. Algül geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Ambulanz für Pankreaserkrankungen, II. Medizinische Klinik und Poliklinik, Klinikum rechts der Isar, Technische Universität München, Ismaninger Straße 22, 81675, München, Deutschland

    S. Rasch, V. Phillip, R.M. Schmid & H. Algül

  2. Institut für allgemeine Pathologie und pathologische Anatomie, Klinikum rechts der Isar, Technische Universität München, München, Deutschland

    G. Weirich & I. Esposito

  3. Institut für diagnostische und interventionelle Radiologie, Klinikum rechts der Isar, Technische Universität München, München, Deutschland

    J. Gaa

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  1. S. Rasch
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  2. V. Phillip
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  3. G. Weirich
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  4. I. Esposito
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  7. H. Algül
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Correspondence to S. Rasch.

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Rasch, S., Phillip, V., Weirich, G. et al. Autoimmune Pankreatitis – Behandlung und Problematik der Diagnosestellung. Internist 55, 1231–1241 (2014). https://doi.org/10.1007/s00108-014-3562-2

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