IgG4-related disease manifesting as an isolated gastric lesion- a literature review

Abstract

Introduction: IgG4-related disease (IgG4-RD) is a newly recognised disorder of unknown etiology and pathogenesis, characterised by dense IgG4+ cells infiltration and fibrosis. IgG4-RD can affect various organs, but gastrointestinal tract involvement is rare. First case of isolated gastric IgG4-RD reported in polish population was diagnosed in our Clinic and became the reason for conducting a literature review. Materials and methods: A literature review was performed using PubMed database. Eight studies of isolated gastric IgG4-RD, published between 2011 and 2017, and a case diagnosed by the authors were included. Results: Three out of nine analysed patients had gastrointestinal complaints. In other cases lesions were detected accidentally. The majority of them were submucosal tumors while only one was a gastric ulcer. The most commonly affected was the stomach body. In all cases malignancy had been suspected, and the lesions were surgically removed. Diagnosis was based on the histopathology image and immunohistochemical staining. Only one patient had elevated IgG4 serum level. No case of recurrence or other organ involvement was reported. Conclusion: IgG4-related disease may manifest as an isolated gastric lesion and should be taken in consideration in differential diagnosis. Making an ultimate diagnosis without histopathological specimen examination seems to be difficult and can lead to misdiagnosis followed by inappropriate treatment. IgG4-RD responds well to steroid therapy. However, on this matter further studies are needed.