Case Report
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World J Gastroenterol. Nov 28, 2014; 20(44): 16782-16785
Published online Nov 28, 2014. doi: 10.3748/wjg.v20.i44.16782
IgG4-related disease manifesting as an acute gastric-pericardial fistula
James Frydman, Shahar Grunner, Yoram Kluger
James Frydman, Shahar Grunner, Yoram Kluger, Department of General Surgery, Rambam Health Care Campus, Haifa 31096, Israel
Author contributions: Frydman J, Grunner S, Kluger Y equally participated in the conception, design, analysis and drafting of this article; Frydman J revised the article critically for important intellectual content; all the authors gave final approval of the version to be published.
Correspondence to: James Frydman, MD, FACS, Department of General Surgery, Rambam Health Care Campus, POB 9602, Haifa 31096, Israel.
Telephone: +972-50-2061886 Fax: +972-4-8543273
Received: March 14, 2014
Revised: May 10, 2014
Accepted: July 24, 2014
Published online: November 28, 2014

IgG4-related disease is a recently recognized entity linked initially to autoimmune pancreatitis and has been subsequently described in nearly every organ system. Men over the age of 50 represent the most affected demographic group and a comprehensive set of diagnostic criteria has been developed to aid treating clinicians. Though elevated levels of IgG4 in the serum are suggestive of the disease, definitive diagnosis is made on histopathology. Treatment is tailored to the clinical presentation with corticosteroid therapy known to have proven efficacy. Gastric manifestations of the IgG4-related disease primarily come in two varieties, notably chronic ulceration or pseudotumor formation. Autoimmune pancreatitis conveys increased risk for IgG4-related disease of the stomach, which is independent of Helicobacter pylori status. In this case report, we present an acute gastric-pericardial fistula secondary to IgG4-related disease that required urgent operative management. To our knowledge, this is the first report in the medical literature describing this complication of IgG4-related disease.

Keywords: IgG4-related disease, Autoimmune pancreatitis, Gastric ulcer

Core tip: IgG4-related disease has been an increasingly recognized entity affecting multiple organ systems. Lesions may mimic neoplasms, yet corticosteroid therapy is highly efficacious. In the stomach, manifestations include ulceration and pseudotumor formation. This case report describing a complication of the disease, notably an acute gastric-pericardial fistula has yet to have been described in the medical literature.