What is Menetrier Disease ( Hypoproteinemic Hypertrophic Gastropathy )?
Menetrier disease is a rare condition known for creating large, abnormal folds in the stomach lining, reduced stomach acid production, and a state in which the body loses protein, leading to low levels of a protein called albumin in the blood. This disease is also referred to as hypoproteinemic hypertrophic gastropathy and giant hypertrophic gastritis.
It’s most often found in men aged between 30 to 60 years, and it’s less commonly seen in women or children. In adults, the disease usually gets progressively worse. The exact causes and development mechanism of Menetrier disease isn’t fully understood, but it mainly affects the upper section of the stomach (body and fundus) while sparing the lower part (antrum).
What Causes Menetrier Disease ( Hypoproteinemic Hypertrophic Gastropathy )?
The exact cause of Menetrier disease is still not fully understood. Nonetheless, many researchers believe that the disease is usually picked up over a lifetime, and not inherited or passed down from parents to children. Interestingly, a temporary form of Menetrier disease is often observed in children when they have contracted a virus known as cytomegalovirus. Similarly, in adults, Menetrier disease has been linked to a bacterial infection caused by Helicobacter pylori.
Furthermore, medical experts believe that the overproduction of a protein, called the transforming growth factor (TGF)-alpha, present in the uppermost layer of the stomach lining, might also contribute to the development of this disease.
Although the disease is not typically inherited, there have been a few rare instances where multiple family members, across several generations, have been diagnosed with Menetrier disease. In one marked case, a mutation in the SMAD4 gene, known to cause juvenile polyposis syndrome, was also suggested as a possible cause of Menetrier disease in a large family spanning four generations. Please remember, these are rare occurrences; in most cases, the disease isn’t inherited.
Risk Factors and Frequency for Menetrier Disease ( Hypoproteinemic Hypertrophic Gastropathy )
Menetrier disease is a rare condition, with less than a thousand cases reported so far. While it can affect anyone, it’s more often found in men and people over 30. The symptoms generally start around the age of 55. According to a study, after diagnosis, 72.7% and 65% of people survived for 5 and 10 years, respectively. However, 8.9% developed stomach cancer within a decade of being diagnosed.
Signs and Symptoms of Menetrier Disease ( Hypoproteinemic Hypertrophic Gastropathy )
Menetrier disease is a condition that gradually appears and progresses in adults. It commonly causes upper stomach pain and is often accompanied by feeling tired, having no appetite, losing weight, swelling (edema), and vomiting. Other symptoms can include nausea, diarrhea, and bleeding from the stomach due to erosion and ulcers. In rare cases, a condition called gastroduodenal intussusception, where one part of the stomach or intestines folds into another part, can occur. Other symptoms linked to losing protein from the intestines (hypoalbuminemia), such as an accumulation of fluid in the abdomen (ascites), fluid in the pleural cavity around the lungs (pleural effusion), and fluid around the heart (pericardial effusion), can also be observed.
In children, Menetrier disease typically starts suddenly and usually gets better on its own within a few weeks. It is often associated with a cytomegalovirus (CMV) infection. In adults, it is usually linked with an H. pylori infection. There is also a weak connection between Menetrier disease and ulcerative colitis, a type of inflammatory bowel disease.
Testing for Menetrier Disease ( Hypoproteinemic Hypertrophic Gastropathy )
A diagnosis can be made through a specific type of tissue sampling, known as a biopsy, which shows exceptional growth of lining cells of the stomach with weakening changes in glands. In addition, a noticeable enlargement of the stomach’s naturally occurring folds can be seen during an esophagogastroduodenoscopy (EGD), a procedure where a thin tube with a light and camera attached to it is used to visualize your esophagus, stomach, and the beginning of your small intestine. During this procedure, large gastric folds, small superficial sores, and redness can be seen.
To confirm, a full-thickness biopsy of the involved area may be taken to identify the loss of deep glandular tissue among other common findings.
An alternative approach involves a procedure where barium, a substance that can show up on X-rays, is swallowed. This study reveals significantly enlarged folds along the larger bend of the stomach. The barium appears diluted, indicative of excessive mucus production which in turn hampers the lining of the stomach.
In cases where needed, contrast-enhanced CT scan – which uses X-rays and a special dye to see inside the body – may show thickening of the stomach lining.
The microscopic examination, called histopathology, carried out on the biopsy material, shows consistent findings of tissue alterations. This includes the organization of glands and excessive growth of the stomach lining cells. Furthermore, there is a considerable reduction of acid-producing cells, widening of glands, increased number of inflammation cells, fluid accumulation, and muscle growth.
When it comes to laboratory tests, a complete blood count – a blood test used to evaluate your overall health and detect a wide range of disorders – will be performed. This includes looking at the number of white blood cells, and measuring the amount of hemoglobin, hematocrit, and platelets in your blood. In addition, doctors will look at the levels of certain substances in your blood, test for a bacteria called H. pylori, and check for cytomegalovirus (CMV), a type of herpesvirus.
Common lab findings include a decrease in the number of lymphocytes (a type of white blood cell), lower levels of certain proteins, cholesterol, fibrinogen (a type of protein in the body), and ceruloplasmin (an enzyme in the body), increased levels of stomach produced hormone called gastrin, and iron deficiency anemia, a condition in which blood lacks enough healthy red blood cells.
Treatment Options for Menetrier Disease ( Hypoproteinemic Hypertrophic Gastropathy )
Menetrier disease treatment isn’t straightforward, largely because we lack many clinical trials to guide us, and medications haven’t consistently shown benefits. However, there are a few different treatment methods that can help manage the disease.
Supportive treatments usually involve a high-protein diet to maintain the body’s protein levels given the disease often leads to protein loss. Medicines called proton pump inhibitors are often used too. They work by reducing the amount of acid your stomach makes, which can help ease symptoms. Along with this, it might be necessary to replace certain micronutrients in your body, as these can also be lost due to the disease.
If tests show you have H. pylori or CMV infections, it’s usually recommended these are treated. H.pylori is a type of bacteria that can cause stomach ulcers, and treating it can sometimes help improve symptoms of Menetrier disease. Ganciclovir is a medication often used to treat CMV infection, and it has been shown to work well in some CMV-related Menetrier disease cases.
In a few isolated reports, patients have also seen benefits from using a drug called octreotide. It works by altering the TGF-alpha – EGFR pathway, which plays a key part in causing Menetrier disease.
There has also been a small study where patients with severe Menetrier disease showed significant improvement after one month of treatment with a drug called cetuximab. Cetuximab works by binding to a specific part of the EGF receptor, preventing TGF-alpha from interacting with it, thereby reducing disease symptoms.
In some cases, surgery may be advised. This is usually if the disease progresses and causes severe symptoms like intense abdominal pain, inability to take food orally, weight loss, bleeding, or blockage of the stomach exit. Partial or total removal of the stomach, a procedure known as a gastrectomy, may be considered in these severe cases. Normally, a total gastrectomy is preferred because it tends to have better results.
What else can Menetrier Disease ( Hypoproteinemic Hypertrophic Gastropathy ) be?
Menetrier disease can be confused with several other conditions due to similar symptoms. The various conditions that should be considered include:
- Zollinger-Ellison syndrome: This condition involves a tumor in the duodenum or pancreas, which leads to overproduction of stomach acid. This can cause peptic ulcers in the GI tract. Through examination of tissue samples, you can see an excessively high number of parietal cells in the stomach.
- H. pylori gastritis: This type of stomach inflammation is clearly present, and can be confirmed with positive H. pylori tests.
- Hypertrophic lymphocytic gastritis: It very much resembles Menetrier disease. When a tissue sample from the stomach is examined, it shows severe inflammation with a lot of lymphocytes within the epithelial layer of cells.
- Hypertrophic hypersecretory gastropathy: With this condition, all different types of cells in the stomach’s glands secrete too much. This results in excessive amounts of stomach acid, pepsin, and mucin. On tissue examination, there’s excessive growth of the uppermost layer of the stomach lining as well as of the glands situated deep within it.
- Gastric polyps: These are more localised as opposed to the diffuse nature of Menetrier disease.
- Gastric adenocarcinoma: Tissue examination from this gastric cancer reveals abnormal growth and development as well as loss of the normal structure, which sets it apart from Menetrier disease.
- Infectious conditions like histoplasmosis, syphilis, tuberculosis, and infiltrative disorders like sarcoidosis: These can present symptoms similar to Menetrier disease.
What to expect with Menetrier Disease ( Hypoproteinemic Hypertrophic Gastropathy )
In kids, this disease normally gets better on its own. It can take several weeks to months for the illness to go away without any medical intervention. With a drug called ganciclovir, children’s symptoms of CMV (a type of virus) infection get better.
In contrast, for adults, the disease tends to get worse over time. It’s rare for adults to fully recover after treating the H. pylori bacterial infection.
People with Menetrier disease, please note, you may be at higher risk for stomach cancers, including gastric carcinoma and gastric lymphoma. Therefore, some doctors recommend regular endoscopic monitoring (a procedure where they look inside your body using a tool called an endoscope) to spot any abnormal cell growth or cancer at its earliest stages.
Possible Complications When Diagnosed with Menetrier Disease ( Hypoproteinemic Hypertrophic Gastropathy )
In adults, this illness progresses over time and can often come with serious health issues and even the risk of death. Generally, problems involve the disease becoming cancerous, the formation of harmful clots, and blockage of the stomach outlet.
People may also experience additional health problems related to surgery on the stomach. These issues can include situations where a connection between two body parts leaks or forms a wrong connection, and instances of bleeding, among others.
Common Complications:
- The disease becoming cancerous
- Formation of harmful blood clots
- Blockage of the stomach outlet
- Leakage or bad connections between body parts after surgery
- Bleeding after surgery
Preventing Menetrier Disease ( Hypoproteinemic Hypertrophic Gastropathy )
Menetrier disease is a seldomly encountered disease, which often causes symptoms like losing weight, feeling sick, throwing up, abdominal pain and weight loss again. There’s no one-size-fits-all treatment that has been proven to work, so it’s important for patients to understand this. A consultation with a dietitian might be beneficial to help patients learn about the need for a diet high in protein.
It’s also crucial for patients to know that this disease can potentially develop into a more serious condition, so it’s recommended that they regularly check in with a specialist doctor for the stomach and intestine, known as a gastroenterologist.
