Immunoglobulin G4 (IgG4)-related disease of the stomach – a challenging differential diagnosis in suspected gastric cancer

Abstract

Background Immunoglobulin G4-related disease (IgG4-RD) can involve different organs and is diagnosed by a combination of clinicopathological features, including storiform fibrosclerosis infiltrated by numerous IgG4-positive plasma cells that frequently forms tumor-like lesions with or without associated obliterative phlebitis. Involvement of the stomach is rare and can occur as part of a multiorgan involvement of IgG4-RD or as isolated gastric involvement.

Case report We report 2 female patients with therapy-refractory gastric ulcers associated with gastric wall thickening and lymphadenopathy that were highly suggestive of gastric cancer or lymphoma. Biopsies failed to confirm a diagnosis, and IgG4-RD was diagnosed only after surgical resection in both patients. The previous literature on gastric IgG4-RD is summarized and shows different characteristics in patients with multiorgan IgG4-RD and isolated gastric IgG4-RD. As reported for autoimmune pancreatitis type 1, patients with multiorgan IgG4-RD are mainly elderly men with frequently elevated serum IgG4 concentrations. In contrast, isolated gastric IgG4-RD predominantly affects female patients with normal serum IgG4 levels. Surgical resection is commonly performed due to the clinical suspicion of malignancy and the absence of findings indicative of IgG4-RD on biopsy. Today, diagnosis is confirmed histopathologically only after resection.

Conclusion IgG4-RD should be taken into account when gastric malignancy is suspected endoscopically or radiologically and biopsies fail to confirm the presence of a malignancy (especially subepithelial tumors or refractory gastric ulcers). Serum IgG4 concentrations are insufficient to confirm localized gastric IgG4-RD. Diagnostic workups need to be improved to avoid unnecessary surgical resections with the attendant potential morbidity and mortality.

Zusammenfasung

Hintergrund Immunglobulin-G4-assoziierte Erkrankungen (IgG4-related diseases, IgG4-RD) werden durch eine Kombination klinischer und histopathologischer Kriterien diagnostiziert (storiforme Fibrosklerose, Infiltration IgG4-positiver Plasmazellen, Bildung von tumor-like lesions, obliterative Phlebitis). Eine gastrale Manifestation ist selten. Bisher sind nur wenige Fallberichte publiziert.

Falldarstellung Wir berichten über zwei Patientinnen mit therapierefraktären Magenulzera, Magenwandverdickung und paragastraler Lymphadenopathie. Wiederholte Biopsien waren nicht wegweisend. Aufgrund des hohen Malignomverdachts erfolgte bei beiden Patientinnen eine Gastrektomie. Erst am chirurgischen Resektat wurde eine IgG4-RD diagnostiziert. Die vorliegende Literatur beschreibt eine gastrale Mitbeteiligung im Rahmen einer Multiorganerkrankung, aber auch Fälle mit isoliertem Magenbefall. Klinisch unterscheiden sich beide Gruppen. Ähnlich der Typ-1-Autoimmunpankreatitis handelt es sich bei Patienten mit Multiorganerkrankung um ältere Männer mit erhöhtem Serum-IgG4. Demgegenüber dominiert bei isoliertem Magenbefall das weibliche Geschlecht. Serum-IgG4 ist bei ihnen nicht erhöht. Die Diagnosestellung erfolgt meist erst nach einer chirurgischen Resektion, die aufgrund eines klinischen Malignomverdachts und unergiebiger Biopsie erfolgt.

Zusammenfassung Eine IgG4-assoziierte Genese sollte als Differenzialdiagnose in Erwägung gezogen werden, wenn endoskopische und radiologische Befunde ein Malignom des Magens vermuten lassen, Biopsien aber nicht wegweisend sind (Magenwandverdickung, refraktäre Magenulzera, Lymphadenopathie). Serum-IgG4-Spiegel sind bei isoliertem Magenbefall nicht verlässlich erhöht. Eine verbesserte Diagnostik muss angestrebt werden, um unnötige chirurgische Resektionen mit ihrer Morbidität und Mortalität zu vermeiden.

Key words

gastric ulcer - IgG4 - IgG4-related disease

Schlüsselwörter

Magenulkus - IgG4 - IgG4-assoziierte Erkrankung

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