Ménétrier disease causes the ridges along the inside of the stomach wall—called rugae—to enlarge, forming giant folds in the lining of the stomach. The rugae enlarge because of an overgrowth of surface mucous cells of the stomach. Ménétrier disease is also called hypoproteinemic hypertrophic gastropathy.
In a normal stomach, rugae release protein-containing mucus. Enlarged rugae release too much mucus, causing a leakage of protein from the blood into the stomach. This shortage of protein in the blood is known as hypoproteinemia. Ménétrier disease also causes a decrease in stomach acid resulting from a reduction in acid-producing parietal cells.
Ménétrier disease is rare and more common in men, usually appearing between the ages of 30 and 60.
What causes Ménétrier disease is unclear; however, it is thought to be an acquired disorder with no known genetic component. Recent studies suggest people with Ménétrier disease have stomachs that make abnormally high amounts of a protein called transforming growth factor-alpha (TGF-α). Growth factors are proteins in the body that tell cells what to do, such as grow larger, change shape, or divide to make more cells. A cause for the overproduction of TGF-α has yet to be found.
People with Ménétrier disease suffer from:
They also have a higher risk of developing stomach cancer, also called gastric cancer.
Treatment may include medications to relieve nausea and pain. A high-protein diet is prescribed to offset the loss of protein from enlarged rugae. Part or all of the stomach may need to be removed if the disease is severe. The anticancer drug cetuximab (Erbitux) blocks the action of TGF-α and is being investigated as a promising new treatment for Ménétrier disease.