Immunoglobulin G4 (IgG4)-related disease of the stomach – a challenging differential diagnosis in suspected gastric cancer
Probst A, Schaller T, Sommer F, Geissler B, Agaimy A, Messmann H, Märkl B (2019)
Publication Type: Journal article
Publication year: 2019
Journal
Book Volume: 57
Pages Range: 1298-1303
Journal Issue: 11
DOI: 10.1055/a-1013-4437
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.
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APA:
Probst, A., Schaller, T., Sommer, F., Geissler, B., Agaimy, A., Messmann, H., & Märkl, B. (2019). Immunoglobulin G4 (IgG4)-related disease of the stomach – a challenging differential diagnosis in suspected gastric cancer. Zeitschrift für Gastroenterologie, 57(11), 1298-1303. https://doi.org/10.1055/a-1013-4437
MLA:
Probst, Andreas, et al. "Immunoglobulin G4 (IgG4)-related disease of the stomach – a challenging differential diagnosis in suspected gastric cancer." Zeitschrift für Gastroenterologie 57.11 (2019): 1298-1303.
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