The diagnosis of IgG4-related disease in these cases was secured on final pathology after laparoscopic wedge resections[17, 18] and endoscopic submucosal resection[19]. IgG4-related disease of the stomach, which is independent ofHelicobacter pyloristatus. 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. == INTRODUCTION == IgG4-related disease was first recognized as a new clinicopathological entity by Kamisawa et al[1] in 2003 when extrapancreatic manifestations were diagnosed with higher frequency in patients with autoimmune pancreatitis (AIP). Multiple organ systems are now known to be affected by the disease, including gastrointestinal, hepatobiliary, pulmonary, genitourinary, cardiovascular, lymphatic, skin, salivary, endocrine and central nervous system[1-9]. Furthermore, with respect to gastric disease, autoimmune pancreatitis is a risk factor for high prevalence of ZJ 43 chronic gastric ulceration, which is independent ofHelicobacter pylori(H. pylori) infection status[10,11]. In this case report, we describe a patient who underwent urgent repair of a gastric-pericardial fistula secondary to chronic gastric ulceration by a dense lymphoplasmacytic infiltrate of IgG4-positive plasma cells. To our knowledge, this complication of IgG4-related disease has not yet been described in the medical literature. == CASE REPORT == The patient is a 65 years old male with a history of tobacco abuse and poor hygiene, who three years prior to the current presentation underwent an emergent antrectomy with Roux-en-Y reconstruction secondary to massive bleeding from erosion of a posterior duodenal ulcer into the gastroduodenal artery (GDA) and penetration into the pancreatic head. The bleeding was arrested by undersuturing of the GDA in four quadrants, and a tube duodenostomy was utilized to manage the difficult stump. Post-operative course was unremarkable and he was discharged home after 10 d. He was followed by the gastroenterology service with findings of a benign marginal ulcer at the gastro-jejunal anastomosis on endoscopy which was treated conservatively, though the patient was poorly compliant and lost to follow-up. At this time he presented to the emergency department with a three day history of ZJ 43 intense upper abdominal and inter-scapular back pain, associated with fever up to 38.4 C. The patient appeared anxious and malnourished. Heart rate was regular at 86 ZJ 43 bpm with blood pressure of 90/50 mmHg. Abdomen was soft, non-distended without peritoneal signs and there was no melena on rectal exam. Laboratory analysis was significant for leukocytosis (WBC = 14.4 103/L with bandemia of 7%), severe anemia (Hgb = 6.7 g/dL) and renal insufficiency (Cr = 1.5 mg/dL). He was found to have a metabolic acidosis (pH = 7.33, Lactate = 9.0 mmol/L), elevated troponin-I (0.3 ng/mL) and nutritional depletion (albumin = 2.2 g/dL). Diagnostic work-up included an abdominal ultrasound which was negative for an abdominal aortic aneurysm or free peritoneal fluid. Chest X-ray was remarkable for pneumopericardium (Figure1). Computed tomography (CT) of the chest demonstrated a large pneumopericardium (Figure2) as well as pneumomediastinum, whose source was a possible fistula emanating from the upper GI tract below the diaphragm (Figure3). Upper gastrointestinal endoscopy (performed without air insufflation secondary to risk of cardiac tamponade) confirmed a large, necrotic gastric ulcer emanating from the Rabbit polyclonal to ASH1 cardia with fistulization toward the pericardium. == Figure 1. == Chest radiograph. Arrow demonstrates air outlining cardiac silhouette. R: Right. == Figure 2. == Computed tomography axial section at thoracic level revealing pneumopericardium, as denoted by arrow. == Figure 3. == Computed tomography axial section at upper abdominal level revealing the ZJ 43 presence ZJ 43 of a gastric-pericardial fistula, as denoted by arrow. After a short period of fluid resuscitation, the patient was brought to the operating room for an exploratory laparotomy. An inflammatory mass was found in the stomach remnant with evidence of fistulization via the diaphragm into the pericardium. A completion gastrectomy was performed with Roux-en-Y esophagojejunostomy and the pericardium was drained. The post-operative course was complicated by respiratory failure requiring percutaneous tracheostomy, as well as treatment for pneumonia and pulmonary embolus. Final pathology revealed a chronic gastric ulcer with extensive fibrosis and chronic inflammatory changes. On immunohistochemistry, multiple IgG4 positive plasma cells were scattered.