Health Questions and Answers

Question: What is Ménétrier’s disease, and how does it differ from the other special forms of chronic gastritis?

Answer:

Ménétrier’s disease differs from the other forms of chronic gastritis in that it is not associated with any significant degree of mucosal inflammation. The disease is seen usually in middle-aged adults. They often present with weight loss, diarrhea, and hypoproteinemic edema. Low acid secretion, loss of parietal cell mass, and protein-losing gastropathy are typical of this disease. The degree of symptomatology varies greatly between patients. Its most severe manifestation is severe wasting secondary to GI protein loss. The endoscopic appearance is that of large folds in the upper stomach, and maximal inflation does not bring about the disappearance of these thick fundal rugae. Diagnosis is by endoscopic biopsy. Loop biopsy from the top of a large fold is usually necessary to obtain adequate tissue for diagnosis. Full-thickness mucosal biopsies show the characteristic massive foveolar hyperplasia seen with this disease. Regular or jumbo forceps biopsies are not adequate for the diagnosis. Endoscopic ultrasound has greatly enhanced the ability of the clinician to differentiate between the various pathologic entities presenting with large or giant gastric folds. This technique defines five distinct layers of the gastric wall. Ménétrier’s disease typically produces thickening in the second layer only, corresponding to the mucosa, whereas carcinoma and lymphoma will also usually involve the third and fourth layers (i.e., the submucosa and muscularis propria). Lymphoma and simple rugal hyperplasia typically involve the second and third layers and can thus be differentiated from Ménétrier’s disease. The treatment of Ménétrier’s disease with H2-blockers, prostaglandins, and proton pump inhibitors has generally been unsatisfactory. High doses of anticholinergics have reduced albumin loss. Subtotal or total gastrectomies have been performed in patients with severe and intractable symptomatology.

Reference: Wolfsen HC, Carpenter HA, Talley NJ: Ménétrièr’s disease: A form of hypertrophic gastropathy or gastritis? Gastroenterology 104:1310-1319, 1993

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