Ménétrier's Disease

A disorder in which enlarged gastric rugae affect part or all of the stomach.

This rare idiopathic disease is difficult to define. It most often occurs in patients > 50. A wide variety of symptoms occur, including epigastric pain, weight loss up to 25 kg (55 lb), nausea, vomiting, gastrointestinal bleeding, and diarrhea. Typical findings include enlarged gastric folds (especially in the fundus and body), histologic features of foveolar hyperplasia and glandular atrophy, and an overall increase in mucosal thickness. Hypoalbuminemia occurs in 20 to 100% of patients (depending on at what stage the disease is diagnosed). The amount of basal and stimulated gastric acid secretion is low or normal. Small or moderate increases in serum gastrin levels may be associated with acid hypersecretion.

The natural history of Ménétrier's disease is not well documented. Symptoms may persist for decades. Although a relationship to stomach cancer was initially described, this association is reported to be only 2 to 15%.

Diagnosis

Diagnosis is suggested when barium swallow demonstrates enlarged, nodular folds. Upper endoscopy and endoscopic ultrasound can confirm the x-ray findings. Definitive diagnosis requires biopsy that reveals marked oxyntic gland loss and cystic changes. However, biopsy may not be necessary, especially in patients with typical clinical features occurring over several years.

Treatment

Information about prognosis is limited. No drug therapy has consistently provided beneficial results. Some patients with associated ulcers or erosions may benefit symptomatically from peptic ulcer therapy. H. pylori, if found, should be eradicated. Surgery, most commonly subtotal gastrectomy, is advocated for patients who have intractable symptoms or in whom a malignancy cannot be excluded.