A Preliminary Study Of Malignancy In IgG4-related Disease

Background: IgG4-related disease is a newly recognized chronic and systemiccondition. In recent years, some literatures have reported cases of IgG4-RDcomplicated with malignancies, and several retrospective studies have been conductedby the researchers from Japan and the United States, however, the relationshipbetween IgG4-RD and malignancy is still unclear.Objective: To retrospectively analyze the clinical features of local IgG4-RDpatients and further explore the possible role and pathogenesis of H.pylori inIgG4-RD and gastric cancer.Method: Twenty-six patients who were diagnosed as IgG4-RD between Januaryof2012and January of2014were analyzed retrospectively in this study.Comprehensive data including gender, age of onset, serological changes and diagnosiswere recorded. Of these26IgG4-RD cases, there was1case of IgG4-RD complicatedwith gastric cancer. His endoscopic biopsy specimens from stomach and resectedgallbladder specimens were examined pathologically via HE staining andimmunohistochemical staining.Result: All of these patients were predominantly middle-aged males, with amean age of60.2±11.2, and the ratio of male to female was2.7:1. Laboratory testsshowed high levels of serum IgG4, with an average concentration of1327.0±947.8mg/dl. All of these26IgG4-RD patients suffered from IgG4-related AIP, in which16cases (61.5%) presented as pancreas involved alone and10cases (38.5%) were incombination with other organ involvement. The multi-organ involvement of these10cases included1case (3.8%) of lacrimal gland involvement,1case (3.8%) of salivarygland involvement,5cases (19.2%) of gallbladder involvement,7cases (26.9%) ofbile duct involvement and1case (3.8%) of lymph node involvement. Of these cases,there was one patient who suffered from IgG4-RD complicated with gastric cancer.His stomach specimens showed abundant infiltration of lymphoplasmacytes andeosinophils in the tumor stroma by HE staining, and the presence of H.pylori in the epithelial cells or cancer cells or mesenchymal cells and numerous infiltration ofIgG-positive or IgG4-positive plasma cells by immunohistochemical staining,however, neither dense fibrosis nor phlebitis was observed. The HE staining of theresected gallbladder specimens revealed abundant infiltration of lymphoplasmacytesand eosinophils as well as fibrosis, and the immunohistochemical staining showed thepresence of H.pylori in the epithelial cells or mesenchymal cells as well as numerousIgG4-positive plasma cells with a ratio of IgG4-positive/IgG-positive plasma cells ofmore than40%.Conclusion: IgG4-related AIP is the predominant manifestation in theseIgG4-RD patients. There was one patient of IgG4-RD complicated withH.pylori-positive gastric cancer. His histopathological changes supported the role ofH.pylori in the initiation of the onset of IgG4-RD and gastric cancer, which maycontribute to the study of the pathogenesis of IgG4-RD and help to explore therelationship between IgG4-RD and malignancy.