Other Name: Hypoproteinemic Hypertrophic Gastropathy
Ménétrier’s disease causes the ridges along the inside of the stomach wall—called rugae—to enlarge, forming giant folds in the stomach lining. The rugae enlarge because of an overgrowth of mucous cells in the stomach wall.
It is a rare, acquired, premalignant disease of the stomach characterized by massive gastric folds, excessive mucous production with resultant protein loss, and little or no acid production. The disorder is associated with excessive secretion of transforming growth factor alpha (TGF-?)
In a normal stomach, mucous cells in the rugae release protein-containing mucus. The mucous cells in enlarged rugae release too much mucus, causing proteins to leak from the blood into the stomach. This shortage of protein in the blood is known as hypoproteinemia. Ménétrier’s disease also reduces the number of acid-producing cells in the stomach, which decreases stomach acid.
The average age of onset is 40 to 60 years, and men are affected more often than women. Adults with Ménétrier disease have a higher risk of developing gastric adenocarcinoma.
Individuals with the disease present with upper abdominal pain (epigastric), at times accompanied by nausea, vomiting, loss of appetite, edema, weakness, and weight loss. A small amount of gastrointestinal bleeding may occur, which is typically due to superficial mucosal erosions; large volume bleeding is rare. 20% to 100% of patients, depending on time of presentation, develop a protein-losing gastropathy accompanied by low blood albumin and edema.
Symptoms and pathological features of Ménétrier disease in children are similar to those in adults, but disease in children is usually self-limited and often follows respiratory infection.
Scientists are unsure about what causes Ménétrier’s disease; however, researchers think that most people acquire, rather than inherit, the disease. In extremely rare cases, siblings have developed Ménétrier’s disease as children, suggesting a genetic link.
Studies suggest that people with Ménétrier’s disease have stomachs that make abnormally high amounts of a protein called transforming growth factor-alpha (TGF-?).
TGF-? binds to and activates a receptor called epidermal growth factor receptor. 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. Researchers have not yet found a cause for the overproduction of TGF-?.
Some studies have found cases of people with Ménétrier’s disease who also had Helicobacter pylori (H. pylori) infection. H. pylori is a bacterium that is a cause of peptic ulcers, or sores on the lining of the stomach or the duodenum, the first part of the small intestine. In these cases, treatment for H. pylori reversed and improved the symptoms of Ménétrier’s disease.1
Researchers have linked some cases of Ménétrier’s disease in children to infection with cytomegalovirus (CMV). CMV is one of the herpes viruses. This group of viruses includes the herpes simplex viruses, which cause chickenpox, shingles, and infectious mononucleosis, also known as mono. Most healthy children and adults infected with CMV have no symptoms and may not even know they have an infection. However, in people with a weakened immune system, CMV can cause serious disease, such as retinitis, which can lead to blindness.
Researchers are not sure how H. pylori and CMV infections contribute to the development of Ménétrier’s disease.
The large folds of the stomach, as seen in Ménétrier disease, are easily detected by x-ray imaging following a barium meal or by endoscopic methods. Endoscopy with deep mucosal biopsy (and cytology) is required to establish the diagnosis and exclude other entities that may present similarly. A non-diagnostic biopsy may lead to a surgically obtained full-thickness biopsy to exclude malignancy. CMV and helicobacter pylori serology should be a part of the evaluation.
Twenty-four-hour pH monitoring reveals hypochlorhydria or achlorhydria, and a chromium-labelled albumin test reveals increased GI protein loss. Serum gastrin levels will be within normal limits.
Other possible causes (eg differential diagnosis) of large folds within the stomach include: Zollinger-Ellison syndrome, cancer, infection (cytomegalovirus/CMV, histoplasmosis, syphilis), and infiltrative disorders such as sarcoidosis.
Cetuximab is the first-line therapy for Ménétrier disease. Cetuximab is a monoclonal antibody against epidermal growth factor receptor (EGFR), and has been shown to be effective in treating Ménétrier disease.
Several medications have been used in the treatment of the condition, with variable efficacy. Such medications include: anticholinergic agents, prostaglandins, proton pump inhibitors, prednisone, and H2 receptor antagonists. Anticholinergics decrease protein loss. A high-protein diet should be recommended to replace protein loss in patients with low levels of albumin in the blood (hypoalbuminemia). Any ulcers discovered during the evaluation should be treated in standard fashion.
Severe disease with persistent and substantial protein loss despite cetuximab may require total removal of the stomach. Subtotal gastrectomy is performed by some; it may be associated with higher morbidity and mortality secondary to the difficulty in obtaining a patent and long-lasting anastomosis between normal and hyperplastic tissue. In adults, there is no FDA approved treatment other than gastrectomy and a high-protein diet. Cetuximab is approved for compassionate use in the treatment of the disease.
Pediatric cases are normally treated for symptoms with the disease clearing up in weeks to months.
Intravenous Protein and Blood Transfusions:
A health care provider may recommend an IV treatment of protein and a blood transfusion to a person who is malnourished or anemic because of Ménétrier’s disease. In most cases of children with Ménétrier’s disease who also have had CMV infection, treatment with protein and a blood transfusion led to a full recovery.
If a person has severe Ménétrier’s disease with significant protein loss, a surgeon may need to remove part or all of the stomach in a surgery called gastrectomy.
Surgeons perform gastrectomy in a hospital. The patient will require general anesthesia. Some surgeons perform a gastrectomy through laparoscopic surgery rather than through a wide incision in the abdomen. In laparoscopic surgery, the surgeon uses several smaller incisions and feeds special surgical tools through the incisions to remove the diseased part of the stomach. After gastrectomy, the surgeon may reconstruct the changed portions of the GI tract so that it may continue to function. Usually the surgeon attaches the small intestine to any remaining portion of the stomach or to the esophagus if he or she removed the entire stomach.
Eating, Diet, and Nutrition:
Researchers have not found that eating, diet, and nutrition play a role in causing or preventing Ménétrier’s disease. In some cases, a health care provider may prescribe a high-protein diet to offset the loss of protein due to Ménétrier’s disease. Some people with severe malnutrition may require IV nutrition, which is called total parenteral nutrition (TPN). TPN is a method of providing an IV liquid food mixture through a special tube in the chest.
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Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.