Idiopathic Hypertrophic Gastropathy – Symptoms, Treatment

Idiopathic Hypertrophic Gastropathy /Menetriers disease (MD) hypoproteinemic hypertrophic gastropathy, is a rare, acquired, premalignant, excessive protein-losing hypertrophic gastropathy disorder characterized by giant mucosal folds in the proximal part of the stomach, thickening of the mucous membrane in the form of giant rugal folds, diminished acid secretion, and a protein-losing state with hypoalbuminemia in giant gastric rugal folds in the body and fundus of the stomach, often with antral sparing, decreased acid secretion, increased gastric mucus production, and hypoalbuminemia and occasional severe upper gastrointestinal hemorrhage.^[rx–rx]secondary to protein loss in the gastric mucosa [rx]. It 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 affects men more frequently than women, and the typical age at diagnosis is between 30 to 60 years.

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.

Another name

• Giant hypertrophic gastritis or
• Hypoproteinemic hypertrophic gastropathy
• Idiopathic hypertrophic gastropathy.
• Rare idiopathic hypertrophic gastropathy.

What Causes Menetriers Disease?

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.

• In children, a self-limited variant of Menetrier disease is observed, which is associated with cytomegalovirus infection.[rx] Similarly, in adults, it is associated with Helicobacter pylori infection.[rx][rx] It is hypothesized that overexpression of the transforming growth factor (TGF)-alpha in superficial gastric epithelium may play a role in etiopathogenesis.[rx]
• Studies suggest that people with Ménétrier’s disease have stomachs that make abnormally high amounts of a protein called transforming growth factor-alpha (TGF-α).
• TGF-α 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-α.
• 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.
• 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.

What are the Signs and Symptoms of Menetriers Disease?

The most common symptom of Ménétrier’s disease is pain in the upper middle part of the abdomen. The abdomen is the area between the chest and hips.

Other signs and symptoms of Ménétrier’s disease may include

• The most common clinical features are epigastric pain (65%), followed by fatigue (60%), anorexia (45%), weight loss (45%), edema (38%), and vomiting (38%).[rx]
• Other symptoms are nausea, diarrhea, and gastrointestinal bleeding related to gastric erosion and ulcers.[rx]
• Rarely a patient may have gastroduodenal intussusception as a presentation.[rx]
• Other clinical features that are associated with hypoalbuminemia from a protein-losing enteropathy like ascites, pleural effusion, and pericardial effusion can also be seen.[rx]
• nausea and frequent vomiting
• diarrhea
• loss of appetite
• extreme weight loss
• malnutrition
• low levels of protein in the blood
• swelling of the face, abdomen, limbs, and feet due to low levels of protein in the blood
• anemia—too few red blood cells in the body, which prevents the body from getting enough oxygen—due to bleeding in the stomach

People with Ménétrier’s disease have a higher chance of developing stomach cancer, also called gastric cancer.

How is Ménétrier’s disease diagnosed?

Health care providers base the diagnosis of Ménétrier’s disease on a combination of symptoms, lab findings, findings on upper gastrointestinal (GI) endoscopy, and stomach biopsy results. A health care provider will begin the diagnosis of Ménétrier’s disease by taking a patient’s medical and family history and performing a physical exam. However, a health care provider will confirm the diagnosis of Ménétrier’s disease through a computerized tomography (CT) scan, an upper GI endoscopy, and a biopsy of stomach tissue. A health care provider also may order blood tests to check for infection with H. pylori or CMV.

Medical and family history

• Taking a medical and family history is one of the first things a health care provider may do to help diagnose Ménétrier’s disease. He or she will ask the patient to provide a medical and family history.

Physical exam

A physical exam may help diagnose Ménétrier’s disease. During a physical exam, a health care provider usually

• examines a patient’s body
• uses a stethoscope to listen to bodily sounds
• taps on specific areas of the patient’s body

Lab test

• Typical laboratory – Laboratory evaluation must include a complete blood count, including a white blood cell (WBC) count, hemoglobin, hematocrit, and platelet counts, a complete metabolic panel, serum gastrin, H. pylori, and CMV serology. Typical laboratory findings include lymphopenia, reduced levels of globulin, albumin, alpha-1-antitrypsin, cholesterol, fibrinogen, and ceruloplasmin, elevated serum gastrin, and iron deficiency anemia.[rx][rx]
• Blood test – A health care provider will take a blood sample that can show the presence of infection with H. pylori or CMV. A blood test involves drawing blood at a health care provider’s office or a commercial facility and sending the sample to a lab for analysis.
• A barium Test – shows markedly enlarged folds along the greater curvature of the stomach, sparing the antrum. Barium is diluted due to excess mucus secretion, which is seen as an impaired mucosal coating. On the contrast-enhanced computed tomography (CT) images, diffuse gastric mucosal thickening is seen.[rx][rx]
• CT scan – CT scans use a combination of x rays and computer technology to create images. For a CT scan, a health care provider may give the patient a solution to drink and an injection of a special dye called contrast medium. CT scans require the patient to lie on a table that slides into a tunnel-shaped device where an x-ray technician takes x rays. An x-ray technician performs the procedure in an outpatient center or a hospital, and a radiologist—a doctor who specializes in medical imaging—interprets them. The patient does not need anesthesia. CT scans can show enlarged folds in the stomach wall.
• Upper GI endoscopy – This procedure involves using an endoscope—a small, flexible tube with a light—to see the upper GI tract, which includes the esophagus, stomach, and duodenum. A gastroenterologist—a doctor who specializes in digestive diseases—performs the test at a hospital or an outpatient center. The gastroenterologist carefully feeds the endoscope down the esophagus and into the stomach. A small camera mounted on the endoscope transmits a video image to a monitor, allowing close examination of the stomach lining. The gastroenterologist also can take a biopsy of the stomach tissue during the endoscopy. A health care provider may give a patient a liquid anesthetic to gargle or may spray an anesthetic on the back of the patient’s throat. A health care provider will place an intravenous (IV) needle in a vein in the arm to administer sedation. Sedatives help patients stay relaxed and comfortable. The test can show enlarged folds in the stomach wall.
• Esophagogastroduodenoscopy – Diagnosis can be reached by obtaining a biopsy showing extreme foveolar hyperplasia with atrophic changes in glandular epithelium along with remarkable enlargement of gastric rugae evident on esophagogastroduodenoscopy (EGD) and barium esophagogram. Endoscopy reveals giant gastric folds, superficial punctate erosions, and erythema. It is recommended to obtain a full-thickness mucosal biopsy of the involved mucosa, which will identify the loss of a deep glandular component along with other typical findings.[rx]
• Biopsy – A biopsy is a procedure that involves taking a piece of stomach tissue for examination with a microscope. A gastroenterologist performs the biopsy at the time of upper GI endoscopy. A pathologist—a doctor who specializes in diagnosing diseases—examines the stomach tissue in a lab. The test can diagnose Ménétrier’s disease by showing changes in the stomach’s mucous cells and acid-producing cells.
• Fluoroscopy – Upper GI fluoroscopy findings include markedly enlarged and tortuous folds in the fundus and body, especially along the greater curvature, with sparing of the antrum barium is diluted due to mucus hypersecretion, leading to impaired mucosal coating 

How is Ménétrier’s Disease Treated?

Treatment may include medications, IV protein, blood transfusions, and surgery.

Medications

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.

•  Antibiotics  – In people with Ménétrier’s disease who also have H. pylori or CMV infection, treatment of the infection may improve symptoms. Health care providers prescribe antibiotics to kill H. pylori. Antibiotic regimens may differ throughout the world because some strains of H. pylori have become resistant to certain antibiotics—meaning that an antibiotic that once destroyed the bacterium is no longer effective. Health care providers use antiviral medications to treat CMV infection in a person with a weakened immune system in order to prevent the serious disease from developing as a result of CMV. Antiviral medications cannot kill CMV; however, they can slow down the virus reproduction.
• Proton pump inhibitors – The supportive treatments that are prescribed are a high-protein diet, proton pump inhibitors, and replacement of micronutrients.
• Cetuximab (Erbitux)  – Health care providers may prescribe the anticancer medication cetuximab (Erbitux) to treat Ménétrier’s disease. Studies have shown that cetuximab blocks the activity of epidermal growth factor receptor and can significantly improve a person’s symptoms, as well as decrease the thickness of the stomach wall from the overgrowth of mucous cells. A person receives cetuximab by IV in a health care provider’s office or an outpatient center. Studies to assess the effectiveness of cetuximab to treat Ménétrier’s disease are ongoing. A health care provider also may prescribe medications to relieve nausea and abdominal pain. Cetuximab is a recombinant IgG1 monoclonal antibody. Cetuximab binds to the extracellular portion of the EGF receptor, which inhibits the binding of TGF-alpha.[rx]
• Ganciclovir – treatment of H. pylori and CMV is recommended if the tests for respective infections are positive. Eradication of H.pylori has been shown to improve disease conditions in a few patients. Similarly, treatment with ganciclovir has been shown to be effective in some CMV-related cases.[rx]
• Octreotide – a somatostatin analog, has shown to be beneficial in case reports. It acts by modulating the TGF-alpha – EGFR pathway, which is the underlying pathogenic mechanism. A dose of 100 to 600 micrograms daily subcutaneously or intravenously or octreotide depot formulation 10 to 60 mg intramuscularly every four weeks has been used.[rx][rx]
• 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.

Surgery

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.

Clinical Trials

The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) and other components of the National Institutes of Health (NIH) conduct and support research into many diseases and conditions.

What are clinical trials, and are they right for you?

Clinical trials are part of clinical research and at the heart of all medical advances. Clinical trials look at new ways to prevent, detect, or treat disease. Researchers also use clinical trials to look at other aspects of care, such as improving the quality of life for people with chronic illnesses. Find out if clinical trials are right for you .

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