Abstract
INTRODUCTION: IgG4 related disease (IgG4-RD) is an immune-mediated condition first described in 2001 as part of autoimmune pancreatitis with subsequent recognition as a systemic condition in 2003. IgG4-RD is characterized by lymphoplasmacytic infiltrates enriched with IgG4-plasma cells that involve different organ systems. The pathogenesis is still being studied. We present a case of gastric IgG4-RD initially suspected to be gastric cancer. CASE DESCRIPTION/METHODS: A 64-year-old woman with a history of Sjogren’s disease, presented with a one-month history of nausea, vomiting, abdominal pain, and poor appetite. Home medication included prednisone 10mg TID. Physical exam was non-contributory. Pertinent lab results showed elevated serum IgG, normal IgG4, elevated ANA and SS-A/Ro antibodies. CT abdomen/pelvis showed diffuse wall thickening in the stomach. EGD showed severe diffuse gastropathy with areas of ischemic tissue and fistula tracts. EUS showed marked submucosal gastric thickening. Endoscopic biopsies showed gastric epithelium with ulceration, inflammation, granulation tissue, and atypical cells, negative for H. Pylori. Endoscopic and EUS FNA biopsies were not diagnostic. The patient then underwent full thickness biopsy which showed IgG patchy dense plasma cells with an increase in IgG4 positive plasma cells. The patient had a complicated course with a gastric leak from the surgical biopsy site that eventually healed with observation and steroid therapy. DISCUSSION: Most cases of IgG4-RD involve 2 or more organs and commonly include pancreas, lymph nodes, bile ducts, salivary glands, lacrimal glands, kidneys, retroperitoneum, & lungs which account for 85% of IgG4-RD. Involvement of the GI tract is rare. Our case represents an example of isolated IgG4-RD of the stomach. Literature review showed 13 case reports of gastric IgG4-RD although most were presenting as a solitary mass treated with surgical therapy. Our case report is unique in that our patient presented with diffuse gastric involvement with the appearance of linitis plastica. It is also interesting because IgG4-RD was previously reported to have an association with Sjogren’s Syndrome (SS) or its subtypes; Mikulicz disease, and Küttner tumor. Our patient did not have a further investigation of her SS. Since IgG4-RD of the stomach could mimic gastric cancer clinically, it is essential to recognize it early with immunohistochemical, pathological testing, clinical correlation, and treatment to avoid the morbidity associated with gastrectomy.
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