Menetrier Disease

Background

• Menetrier disease is a rare stomach disease characterized by diffuse hyperplasia of gastric surface mucosal glands, often with antral sparing. It classically presents as a triad of gastrointestinal symptoms, peripheral edema, and thickened gastric folds.
• Menetrier disease has a peak incidence in the fourth to sixth decade of life and may be associated with certain infections including Helicobacter pylori, cytomegalovirus, herpes simplex, and mycoplasma.
• Menetrier disease is caused by overexpression of transforming growth factor alpha, which ultimately leads to foveolar mucous cell hyperplasia and parietal cell inhibition. This results in a reduction of gastric acid and increased mucin production, contributing to malabsorption of nutrients, electrolytes, and vitamins.

Evaluation

• Consider Menetrier disease in patients with symptoms of weight loss, epigastric pain, anorexia, nausea, vomiting, or upper gastrointestinal (GI) bleed (rare) with associated peripheral edema who have large gastric folds on gastric imaging.
• Imaging modalities to assess for large gastric folds include barium studies, computed tomography (CT) scanning, or upper endoscopy.
• Diagnosis is confirmed by deep mucosal biopsies taken during upper endoscopy.
• Assess patients with Menetrier disease for:
  • Gastric mucosal infection by Helicobacter pylori or cytomegalovirus.
  • Hypochlorhydria by sampling gastric acid during endoscopy.
  • Serum albumin level and consider measurement of other nutrient levels.
• Consider measurement of serum gastrin to assess for Zollinger-Ellison syndrome/gastrinoma as a cause of hypertrophic folds.

Management

• Given rare incidence of disease, management recommendations are based on case reports and small series of patients.
• Hypoalbuminemia may be palliated by a high-protein diet.
• In severely hypoalbuminemic patients, parenteral albumin infusion may be useful.
• Helicobacter pylori eradication is recommended, if applicable.
• Consider cetuximab (monoclonal antibody against epidermal growth factor receptor) although there is limited evidence of a benefit. Other agents which have been reported to have some benefit in the treatment of Menetrier disease include:
  • Anticholinergics
  • Prostaglandins
  • Glucocorticoids
  • Octreotide
• Consider endoscopic therapy if there is associated gastrointestinal bleeding.
• Total gastrectomy may be appropriate for intractable symptoms.