ZES should be suspected in patients with ulcers who are not infected with H. pylori and have no history of NSAID use. Diarrhea may precede ulcer symptoms. Ulcers occurring in the second, third, or fourth portions of the duodenum or the jejunum (the middle section of the small intestine) are signs of the syndrome. Gastroesophageal reflux disease (backflow of the stomach's contents into the esophagus) is more prevalent and often more severe in patients with ZES, and can be complicated by ulcerations and strictures of the esophagus.
Peptic ulcers associated with ZES are typically persistent and difficult to treat. Treatment consists of removing the tumors and suppressing acid with intravenous proton-pump inhibitors (Protonix). Proton-pump inhibitors block acid production and are a major advance for these patients. Previously, removing the stomach was the only option.