Last updated:
2/25/2026
Years published: 2006, 2010, 2017, 2026
NORD gratefully acknowledges Gioconda Alyea, MD (FMG), MS, National Organization for Rare Disorders, for the preparation of this report.
Menetrier disease is a rare disorder of the stomach in which the lining of the stomach becomes abnormally thickened. This happens because certain mucus-producing cells in the stomach lining, called foveolar cells, grow in excessive numbers. These cells normally produce mucus that helps protect the stomach from digestive acids.
In Menetrier disease, the overgrowth of these mucus-producing cells causes the inner surface of the stomach to form large, thick folds, which can interfere with normal stomach function.
People with Menetrier disease often experience pain or discomfort in the upper central part of the abdomen, an area known medically as the epigastric region. Other symptoms may include nausea, vomiting, reduced appetite, early fullness when eating, weight loss, and swelling (edema) of the legs or ankles. Swelling occurs due to the loss of protein from the thickened stomach lining, a condition known as protein-losing gastropathy, which lowers blood protein levels and allows fluid to leak into the tissues.
Researchers believe that Menetrier disease is caused by many factors. Increased activity of a protein called epidermal growth factor receptor (EGFR) may lead to excessive growth of mucus-producing cells. In some children, the condition has been linked to infection with cytomegalovirus (CMV) and in adults, some cases have been associated with infection by Helicobacter pylori, a bacterium that commonly infects the stomach.
Treatment is individualized and may include several medications and, in selected and severe cases, surgery to remove the stomach.
Menetrier disease (Ménétrier disease) was first described by the French pathologist Pierre Ménétrier in 1888, hence the name of the disease.
A diagnosis of Menetrier disease is reserved for people whose large gastric folds result from overgrowth of mucus-producing cells, with little or no inflammation. Because inflammation is minimal or absent, Menetrier disease is classified as a form of hyperplastic gastropathy (abnormal overgrowth of the stomach lining) rather than a form of gastritis, which is characterized by an inflammation of the stomach lining.
Medical literature has used the term Ménétrier disease inconsistently, sometimes to describe a group of conditions that involve enlarged folds in the stomach lining. While Menetrier disease and giant hypertrophic gastritis are usually considered synonyms, some researchers believe that Menetrier disease and giant hypertrophic gastritis represent different forms of the same underlying disorder.
The signs and symptoms of Menetrier disease may vary from case to case. Some individuals may not exhibit any symptoms (are asymptomatic).
The most common symptom is pain in the upper middle region of the stomach (epigastric pain). Less frequently reported symptoms include:
Some people with Menetrier disease develop protein-losing gastropathy, a condition in where the stomach’s enlarged, thick mucosal folds leak excessive amounts of serum proteins into the gastrointestinal tract. This may result in:
Other reported signs and symptoms of Menetrier disease may include:
People with Menetrier disease may have a higher risk of developing gastric adenocarcinoma (a type of stomach cancer) than the general population. Because of this increased risk, Menetrier disease is often considered a premalignant condition, meaning it can increase the chance of cancer developing over time. As a result, people with Menetrier disease may be advised to have regular medical follow-up and periodic endoscopic exams (usually an upper endoscopy, a procedure in which a thin, flexible tube with a camera is passed through the mouth into the stomach), which allows doctors to closely monitor the stomach lining.
Associations between Menetrier disease and inflammatory bowel disease, including ulcerative colitis, have been reported but are rare.
The cause of Menetrier disease is not fully understood. Researchers believe the disorder is multifactorial, meaning that more than one factor may contribute to its development.
Studies suggest that the overactivation of the epidermal growth factor receptor (EGFR) is essential for the development of the disease. EGFR is a protein found on the cells that line the surface of the stomach that helps control normal cell growth and repair. In people with Menetrier disease, EGFR signaling is abnormally increased due to an excessive production of the transforming growth factor-alpha (TGF-α), a naturally occurring protein that binds to and activates EGFR.
Excessive EGFR stimulation causes overgrowth (hyperplasia) of the mucus-producing stomach lining cells called foveolar cells (foveolar hyperplasia). This abnormal cell growth leads to thickened folds in the stomach lining and increased mucus production. At the same time, increased EGFR activity reduces the function of acid-producing cells, leading to low stomach acid levels (hypochlorhydria) or no stomach acid at all (achlorhydria). Despite low acid levels, blood levels of gastrin, a hormone that normally stimulates acid secretion, are often normal or only mildly increased.
Abnormal TGF-α–EGFR signaling causes the stomach lining to become thickened and inflamed and can disrupt tight junctions, the structures that normally hold stomach lining cells tightly together and maintain a strong barrier between the bloodstream and the stomach. Under normal conditions, blood proteins do not enter the stomach. However, when this barrier is damaged, proteins such as albumin can leak out of blood vessels in the stomach wall, pass through the weakened stomach lining, and enter the stomach lumen. Once in the stomach, these proteins are lost as they move through the gastrointestinal tract. This protein loss, known as protein-losing gastropathy, leads to low blood protein levels (hypoalbuminemia) and can cause fluid buildup in tissues (edema), especially in the legs and ankles.
In children, many reported cases of Menetrier disease have been associated with cytomegalovirus (CMV) infection. CMV is a common virus that usually causes mild or no symptoms in healthy individuals but may affect the stomach lining in some children. Research suggests that CMV infection may trigger an increased production of TGF-α, leading to activation of the EGFR signaling pathway and the characteristic stomach changes seen in Menetrier disease. In children, CMV-associated Menetrier disease is often self-limited, meaning that symptoms may improve or resolve over time.
In adults, Helicobacter pylori (H. pylori) infection has been identified in some cases of Menetrier disease. H. pylori is a bacterium that commonly infects the stomach and is known to cause chronic gastritis and ulcers. In some individuals with Menetrier disease, treatment to eliminate H. pylori results in the improvement of symptoms of the disease and stomach tissue changes, suggesting the infection may contribute to disease development in susceptible individuals. Laboratory studies indicate that H. pylori may enhance EGFR signaling by increasing TGF-α production and increasing levels of EGFR and related growth factors in the stomach lining. However, a direct cause-and-effect relationship between H. pylori infection and Menetrier disease has not been clearly proven.
Other infectious agents have been reported rarely in association with Menetrier disease, particularly in children. These include herpes simplex virus, Giardia lamblia (a parasitic infection), and Mycoplasma pneumoniae (a bacterial infection). These infections are considered uncommon potential triggers, not established causes.
Rare familial cases of Menetrier disease have been reported, including cases among siblings and across multiple generations. In these families, the condition appears to follow an autosomal dominant inheritance pattern.
Genetic conditions are determined by two copies of each gene, one inherited from each parent. In autosomal dominant disorders, only one altered gene copy is needed for the condition to develop. The altered gene may be inherited from an affected parent, or it can arise from a new gene change (de novo) in the affected individual. When a parent has an autosomal dominant condition, the risk of passing the abnormal gene to offspring is 50% for each pregnancy, regardless of the sex of the resulting child.
Menetrier disease is very rare, and how often it occurs in the general population is unknown due to limited data. As of 2025, fewer than 1,000 cases have been reported worldwide. The disease most often affects adults between 40 and 60 years of age and occurs more frequently in men than in women.
In children, reported cases of Ménétrier disease (pediatric form) are often linked to cytomegalovirus (CMV) infection and usually have a more favorable outcome, with many children improving over time.
Menetrier disease may be suspected in people who are found to have large folds in the stomach lining. Because the condition is rare and no single diagnostic test can confirm it, doctors make the diagnosis by combining information from symptoms, imaging studies, endoscopic exams, laboratory tests, and examination of stomach tissue under a microscope.
Diagnosis is most often made using upper endoscopy, a procedure in which a thin, flexible tube with a camera is passed through the mouth into the stomach. This allows doctors to directly view the stomach lining and obtain biopsy samples, which are small pieces of tissue. In Menetrier disease, endoscopy usually shows unusually thick stomach folds, most often in the upper part of the stomach, while the lower part is usually not affected. During the procedure, doctors may also measure stomach acid levels, which are often low or absent in this condition.
Imaging studies, such as endoscopic ultrasound, CT (computed tomography) scans, or MRI (magnetic resonance imaging), may be used to look at the thickness of the stomach wall and to check for complications such as fluid buildup in the abdomen. Blood tests often show low levels of protein, especially albumin, due to loss of protein through the stomach. Testing for Helicobacter pylori (H. pylori), a stomach bacterium, and cytomegalovirus (CMV), a common virus, is recommended because these infections may be associated with the disease.
A definite diagnosis requires microscopic examination of stomach tissue, a process called histopathology. Typical findings include overgrowth of mucus-producing cells, loss of acid-producing glands, and little or no inflammation. Other conditions that cause thickened stomach folds must be ruled out.
Treatment
There is currently no single standardized treatment for Menetrier disease. Treatment is individualized and depends on the severity of symptoms, the extent of protein loss, and contributing factors such as infection. Because the condition is rare, there are limited clinical trials, and many treatments have shown variable and sometimes inconsistent effectiveness. As a result, treatment focuses on symptom relief, nutritional support, and prevention of complications.
Supportive care is an important part of treatment. Many people benefit from a high-protein diet to help replace protein lost through the stomach. Replacement of vitamins and minerals may also be recommended. In cases of severe protein loss, albumin infusions may be used to temporarily increase blood protein levels and reduce swelling.
If testing shows infection with Helicobacter pylori (H. pylori) or cytomegalovirus (CMV), treatment directed at these infections is recommended. Eradication of H. pylori has been associated with improvement in symptoms and stomach changes in some adults. In selected cases of CMV-associated Menetrier disease, particularly in children, antiviral medications such as ganciclovir may be effective. Many affected children, however, improve over time with supportive care alone.
Several medications have been used to manage symptoms. Proton pump inhibitors, which reduce stomach acid, may help relieve symptoms even though stomach acid production is often already low. Antihistamines and anticholinergic medications have been tried in the past, but they are generally not considered as the first options of treatment, as there is limited evidence that they significantly improve the disease.
Octreotide, a medication that mimics the natural hormone somatostatin, has shown benefit in some individuals with Menetrier disease. Octreotide is thought to work by reducing abnormal growth signaling in the stomach lining, particularly involving transforming growth factor-alpha (TGF-α) and the epidermal growth factor receptor (EGFR). Case reports have described improvement in symptoms, stomach appearance, and microscopic findings after several months of treatment, though dosing and duration are not standardized.
The most promising targeted (disease-directed) therapy to date is cetuximab (Erbitux), a medication that blocks the epidermal growth factor receptor (EGFR). By preventing EGFR activation, cetuximab addresses a key mechanism believed to drive the disease. Studies have shown rapid and sustained improvement in symptoms, normalization of blood protein levels, reduction in swelling, weight gain, and partial restoration of normal stomach structure. In some individuals, stomach acid production improves and abnormal tissue changes nearly resolve.
Cetuximab is generally well tolerated but can cause side effects, including infusion reactions, skin rash, low magnesium levels, and rare heart or lung complications. Careful monitoring by experienced healthcare providers is required. In some individuals, symptoms have returned after stopping treatment, and long-term or ongoing therapy may be considered, but the long-term safety and effectiveness of continuous cetuximab therapy are still being studied. Other targeted therapies that act on the same growth signaling pathway are being investigated and may offer future treatment options.
In individuals with severe disease that does not respond to medical therapy, especially those with persistent and debilitating protein loss, severe symptoms, or concern for cancer development, surgical removal of the stomach (gastrectomy) may be necessary. Although Menetrier disease primarily affects the upper and middle portions of the stomach, total gastrectomy is often preferred over partial removal, as it may lead to better long-term outcomes, fewer complications, and elimination of future cancer risk and the need for ongoing stomach surveillance.
The outlook for Menetrier disease varies. Children often have a favorable prognosis, with many cases resolving over time. In adults, the disease may follow a progressive course and can lead to severe malnutrition. Many experts advise annual upper endoscopy to monitor for disease progression or early signs of stomach cancer.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
TEXTBOOKS
Merchant JL. Giant Hypertrophic Gastritis. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:341.
JOURNAL ARTICLES
Fiske WH, et al. Efficacy of cetuximab in the treatment of Menetrier’s disease. Sci Tranl Med 2009; 1(8): 8ra18.
Hoffer V, et al., Ganciclovir treatment in Menetrier’s disease. Acta Paediatr. 2003;92:983-5.
Dykes CM. Menetrier’s disease: case study in the quality of life. Gastroenterol Nurs. 2003;26:3-6.
Di Vita G, et al., Resolution of Menetrier’s disease after Helicobacter pylori eradicating therapy. Dig Dis. 2001;19:179-83.
Rimar Y, Jaffe M, Shaoul R. Harefuah. 2001;140:586-7, 679.
Burdick JS, et al., Treatment of Menetrier’s disease with a monoclonal antibody against the epidermal growth factor receptor. N Engl J Med. 2000;343:1697-701.
Chang KW, et al., Menetrier’s disease associated with cytomegalovirus infection in a child. Acta Paediatr Taiwan. 2000;41:339-40.
Raderer M, et al., Successful symptomatic management of a patient with Menetrier’s disease with long-term antibiotic treatment. Digestion. 1999;60:358-62.
Kindermann A, Koletzko S. Protein-losing giant fold gastritis in childhood ? a case report and differentiation from Menetrier disease of adulthood. Z Gastroenterol. 1998;36:165-71.
Wolfsen C, Carpenter HA, Talley NJ. Menetrier’s disease: a form of hypertrophic gastropathy or gastritis? Gastroenterology. 1993;104:1310-9.
Sachs M, Encke A. Menetrier disease? A Rare Disease of the Stomach. Zentralbl Chir. 1993;118:160-5.
Meuwissen SG, et al., Hypertrophic protein-losing gastropathy. A retrospective analysis of 40 cases in the Netherlands. The Dutch Menetrier Study Group. Scand J Gastroenterol Suppl. 1992;194:1-7.
Mosnier JF, et al., Hypertrophic gastropathy with gastric adenocarcinoma: Menetrier’s disease and lymphocytic gastritis? Gut. 1991;32:1565-7.
Almazar AE, Penfield JD, Saito YA, Talley NJ. Survival Times of Patients With Menetrier’s Disease and Risk of Gastric Cancer. Clin Gastroenterol Hepatol. 2021;19(4):707-712. doi:10.1016/j.cgh.2020.03.017
Zhang T, Chen T, Tang X. Ménétrier’s Disease: A Narrative Review of Molecular Pathogenesis, Clinical Spectrum, and Evolving Therapeutic Strategies. QJM. Published online November 12, 2025. doi:10.1093/qjmed/hcaf276
Barros AR, Monteiro S, Silva J. Ménétrier disease: A clinical review. World J Gastroenterol. 2025;31(32):108401. doi:10.3748/wjg.v31.i32.108401
INTERNET
Feldman M and Jensen PJ. Large gastric folds: Hyperplastic and nonhyperplastic gastropathies. UpToDate. Last updated: Jul 24, 2025. https://www.uptodate.com/contents/large-gastric-folds-hyperplastic-and-nonhyperplastic-gastropathies?source=see_link Accessed February 2, 2026.
McKusick VA., ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No:137280; Last Update: 09/12/2007. Available at: https://www.omim.org/entry/137280?search=137280&highlight=137280 Accessed February 2, 2026.
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