S3620 IgG4 Gastropathy: Hemorrhagic Gastropathy as the Culminating Presentation of Undiagnosed IgG4-Related Disease

Abstract

Introduction: IgG4 related disease (IgG4-RD) is a newly recognized, immune-mediated disease with IgG4-positive plasma cell infiltration. Serum IgG4 levels are often elevated (although not required for diagnosis). IgG4-RD can affect a multitude of organs, including but not limited to the pancreatic-biliary system, salivary/lacrimal glands, retroperitoneum, liver, aorta, lymph nodes, and rarely, the gastrointestinal tract. This case highlights the rare GI tract presentation of a patient’s heretofore unrecognized IgG4 disease. Case Description/Methods: An 81-year-old woman with past medical history of Sjogren’s disease, myelodysplastic syndrome, and chronic myelomonocytic lymphoma presented to the emergency department for melena. Physical exam was unremarkable, but melena confirmed on digital rectal exam. Laboratory studies with hemoglobin nadir at 5.0 g/dL (baseline 8), hypergammaglobulinemia (without monoclonal peak) and elevated IgG4. Gastroenterology was consulted and performed esophagogastroduodenoscopy, showing hemorrhagic gastropathy and friable tissue. Biopsy showed gastric mucosa with patchy severe chronic gastritis and predominant plasma cell infiltration (without light chain restriction), thought to be isolated IgG4 gastropathy. Melena resolved with supportive care. Bone marrow biopsy obtained, appropriately decreased cellularity, no monocytes on flow cytometry. Abdominal computerized tomography with intravenous contrast showed diffusely atrophic pancreas, 4.8 cm infrarenal aneurysmal abdominal aorta (AAA), and post cholecystectomy changes. Reported history of pancreatitis (records unavailable), fine needle aspiration of pancreatic head and tail (ten years prior) showed predominantly acute inflammatory cells, necrotic tissue debris and benign ductal epithelial cells, respectively. Salivary gland biopsies (ten years prior) showed benign mucinous gland tissue and multiple reactive lymphoid follicles. The clinical picture was concerning for possible systemic IgG4 disease. Unfortunately, four months after initial evaluation, patient suffered from a cerebrovascular event and passed away a month later after progressive debilitation. Discussion: On initial evaluation, patient appeared to present with isolated IgG4 gastropathy. Given history of salivary gland disease, pancreatic disease, and AAA, high degree of suspicion exists for underlying IgG4- RD connecting previous diagnoses. Although rare, IgG4-RD may present in various ways, and clinicians must remain vigilant for appropriate diagnosis and treatment.