IgG4-assoziierte Autoimmunpankreatitis und Lungenerkrankung mit der primären, klinischen Präsentation eines pulmonal metastasierten Pankreaskarzinoms

Zusammenfassung

Hintergrund: Die Autoimmunpankreatitis (AIP) wurde 1995 erstmalig konzeptuell beschrieben und erfolgreich mit Steroiden behandelt. Inzwischen werden 2 histopathologische und klinische Subtypen der AIP unterschieden. Die AIP Typ 1 stellt die pankreatische Manifestation einer Immunglobulin-G4(IgG4)-assoziierten, sklerosierenden Systemerkrankung dar. Die AIP Typ 2 ist IgG4-unabhängig. Für beide AIP-Formen stellt das Pankreaskarzinom die wichtigste Differenzialdiagnose dar.

Fallbericht: Wir berichten über einen 82-jährigen Patienten mit Verschlussikterus, der sich mit dem Bild eines pulmonal metastasierten Pankreaskarzinoms bei uns vorstellte. Nach Gallengangsdrainage mittels ERCP wurde eine Lungenbiopsie durchgeführt, die keinen Tumornachweis erbrachte. Nach Anbehandlung des Patienten unter palliativer Indikation kam es zu einer kompletten Rückbildung der Erkrankung. Die nachträglich durchgeführte, immunhistochemische Färbung des Lungenbiopsats führte zur Diagnose einer IgG4-assoziierten, sklerosierenden Systemerkrankung. Nach Absetzen der Steroidtherapie kam es zu einem Rezidiv der Erkrankung in Form einer sklerosierenden Cholangitis.

Schlussfolgerung: Die IgG4-assoziierte, sklerosierende Systemerkrankung mit Typ1-AIP kann sich bildmorphologisch und klinisch wie ein pulmonal metastasiertes Pankreaskarzinom präsentieren. Eine extrapankreatische IgG4-positive Histopathologie sowie das rasche Ansprechen auf eine Steroidtherapie können helfen, das komplexe Erkrankungsbild zu diagnostizieren.

Abstract

Background: After the first case publication using the term “autoimmune pancreatitis” in 1995 and the successful treatment with steroids we now can distinguish between two clinical und histopathological forms of autoimmune pancreatitis. Type 1 autoimmune pancreatitis (AIP) is usually part of an IgG4-related systemic disease. AIP Typ 2 is an IgG4-independent pancreatic disease. For both entities pancreas cancer is the most important differential diagnosis.

Case Report: We report the case of an 82-year-old male patient who primarily presented with obstructive jaundice. Computed tomography (CT) revealed the typical image of a small cancer of the head of the pancreas with pulmonary metastases. After endoscopic drainage of the bile duct a CT-guided biopsy of a pulmonary nodule was performed in which cancer was ruled out. Next the patient was treated with steroids because of “tumour-associated cachexia”. In the follow-up the mass in the head of the pancreas like the lung nodules had surprisingly disappeared. In the complete work-up the immune histochemical staining of the lung biopsy revealed subsequently a typical IgG4-associated inflammation. After termination of the therapy the disease relapsed as sclerosing cholangitis.

Conclusion: The IgG4-related systemic disease with AIP can present as cancer of the pancreas with lung metastases. Extrapancreatic IgG4-positive histopathology and response to therapy with steroids can help to diagnose the disease in complex clinical presentations.

Schlüsselwörter

Immunglobulin-G4(IgG4)-assoziierte Systemerkrankung - Autoimmunpankreatitis (AIP) mit Lungenerkrankung - pulmonal metastasiertes Pankreaskarzinom

Key words

IgG4-related systemic disease - autoimmune pancreatitis and lung involvement - pancreatic cancer with pulmonary metastasis

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