Ménétrier Disease (also known as giant hypertrophic gastritis or hyperplastic hypersecretory gastropathy) is a rare, acquired condition characterized by the overgrowth of the mucous cells in the lining of the stomach, leading to large, thickened folds in the stomach’s inner lining
About
- Premalignant condition and 15% develop gastric cancer
Aetiology
- Rugal fold hypertrophy and Hyperplasia of protein containing mucus producing cells
- Hypochlorhydria and Protein losing enteropathy
- Releases large amounts of transforming growth factor-alpha (TGF-a).
- May be a role for Helicobacter and CMV
Clinical
- Abdominal Pain: Often located in the upper abdomen.
- Nausea and Vomiting: Common gastrointestinal symptoms associated with the disease.
- Oedema: Swelling, particularly in the lower extremities, due to protein loss (protein-losing gastropathy).
- Weight Loss: Unintentional weight loss is a common symptom.
- Loss of Appetite: Reduced desire to eat, contributing to weight loss.
- Anaemia: Resulting from chronic gastrointestinal blood loss.
Investigations
- Endoscopy: The primary diagnostic tool, which shows thickened gastric folds, particularly in the fundus and body of the stomach.
- Biopsy: taken during endoscopy reveal characteristic findings, including foveolar hyperplasia and glandular atrophy.
- Imaging: CT scans or upper gastrointestinal series may show enlarged gastric folds.
- Laboratory Tests: Blood tests may reveal hypoalbuminemia (low levels of albumin) due to protein loss, and anaemia.
Management
- Medications:
- Proton Pump Inhibitors (PPIs): To reduce gastric acid secretion and manage symptoms.
- Octreotide: A somatostatin analog that can reduce protein loss and improve symptoms.
- Nutritional Support: High-protein diets or intravenous albumin may be necessary to address protein loss.
- Therapeutic Plasma Exchange: In severe cases, to remove circulating growth factors that may contribute to the disease.
- Surgery: Partial or total gastrectomy may be considered in refractory cases or when there is a high risk of malignancy.
References