Treatment of Ménétrier's Disease with a Monoclonal Antibody against the Epidermal Growth Factor Receptor

Abstract
MÉnÉtrier's disease (hypoproteinemic hypertrophic gastropathy) is a rare, acquired, premalignant disorder of the stomach.1-4 It is characterized by giant hypertrophic folds that most often involve the fundus, excess mucus secretion, decreased acid secretion (hypochlorhydria), and hypoproteinemia due to selective loss of serum proteins across the gastric mucosa.5,6 The cause of Ménétrier's disease is unknown, although infection with cytomegalovirus (CMV) in children and infection with Helicobacter pylori have been implicated.7,8 Symptoms include epigastric pain, vomiting, edema, anorexia, and weight loss. Gastric cancer has been reported at diagnosis or during follow-up in patients with hypertrophic gastropathy. Evidence of the benefits of anticholinergic drugs, acid suppression, octreotide, and eradication of H. pylori is inconsistent.2,9,10 Except in cases of CMV-associated Ménétrier's disease in children,11 spontaneous remissions are rare. Partial or total gastrectomy is generally reserved for patients with debilitating disease and for cases in which there is concern over the development of gastric cancer.12