Ménétrier Disease

Ménétrier, in his original description, included patients with multiple hyperplastic polyps and patients with giant folds and focused his observa­tions on the increased gastric cancer risk of these patients. Today, the condition associated with his eponym is defined as an idiopathic diffuse enlargement of the gastric folds in the antrum. Histologically, glands show massive foveolar cell hyperplasia with normal or slightly reduced numbers of parietal and chief cells. Neither significant inflammatory infiltrates in the lamina propria nor epithelial lesions (erosions, intestinal metaplasia, or cellular atypia) are present. If chronic active inflammation or lymphocytic infiltration of the epithelium is seen, the large-fold type of H.pylori gastritis or lymphocytic gastritis should be considered. In children, giant folds are almost always associated with CMV infection. This association has rarely been reported in adults. The hyperplastic foveolar cells secrete large amounts of mucus and fluid resulting in protein-losing enteropathy (found in almost all patients) and hypoacidity (in part from dilution of the acid produced by oxyntic cells).

Pure Ménétrier disease is a rare condition, with an estimated prevalence of no more than 300 or 400 persons worldwide. Patients are more often men in their fifth or sixth decade who present with dramatic weight loss, epigastric and abdominal pain, nausea, and vomiting. Later in the course of the disease, usually protracted for several years and even decades, virtually all patients develop hypoalbuminemia as a consequence of the protein-losing enteropathy. The pathogenesis is unknown, but an interesting hypothesis has been put forward. Transforming growth factor-a (TGF-a), a critical mediator of gastric mucosal homeostasis normally produced by the gastric mucosa, inhibits acid secretion, stimulates mucosal repair, cell migration, and proliferation, and augments gastric mucin levels Overproduction of TGF-a could explain several of the disturbances occur­ring in Ménétrier disease. Support for this hypothesis is further lent by the successful treatment of some patients by a monoclonal antibody against the TGF-a receptor (the epidermal growth factor receptor). Other forms of treatment, including antacids, anticholinergic drugs, corticosteroids, H 2-receptor antagonists, and proton pump inhibitors, have been proven consistently ineffective. Eradication of H.pylori has been reported to have successfully cured patients with Ménétrier disease. One of the possible pathological aspects of H.pylori gastritis is the development of large, edematous folds, and this variant may have been interpreted as a type of H.pylori–induced Ménétrier disease. Not surprisingly, it regresses after the eradication of H.pylori infection.

Дата добавления: 2015-11-14