We're celebrating
40 years!
Last updated:
July 31, 2017
Years published: 2006, 2010, 2017
NORD gratefully acknowledges Mark Feldman, MD, William O. Tschumy Jr. Chair, Department of Internal Medicine and IM Residency Program Director, Texas Health Presbyterian Hospital Dallas, for assistance in the preparation of this report.
Menetrier disease is a rare disorder characterized by massive overgrowth of mucous cells (foveola) in the mucous membrane lining the stomach, resulting in large gastric folds. The most common symptom associated with Menetrier disease is pain in the upper middle region of the stomach (epigastric pain). The cause of Menetrier disease is unknown.
There is considerable confusion and contradiction in the medical literature regarding disorders involving large gastric folds. The name Menetrier disease is often erroneously used to describe any condition with large gastric folds or as a synonym for giant hypertrophic gastritis (GHG). However, Menetrier disease is not a true form of gastritis. A diagnosis of Menetrier disease should be reserved for individuals with large gastric folds due to overgrowth of mucous cells. There is minimal or no stomach inflammation in Menetrier disease. Because inflammation is minimal or not present, Menetrier disease is classified as a form of hyperplastic gastropathy and not as a form of gastritis. Some researchers believe that Menetrier disease and GHG may be variants of the same disorder or different parts of one disease spectrum.
The symptoms of Menetrier disease may vary from case to case. Some individuals may not exhibit any symptoms (asymptomatic). The most common symptom is pain in the upper middle region of the stomach (epigastric pain). Less frequent reported symptoms include nausea, vomiting, and diarrhea. In some cases, weight loss and profound loss of appetite (anorexia) may also occur.
An additional variable finding sometimes associated with Menetrier disease is the loss of protein from the circulation into the gastrointestinal tract (protein-losing gastropathy) such as the loss of the protein albumin (hypoalbuminemia). Protein loss may be severe in some cases. Protein loss may result in fluid accumulation (edema), especially in the legs.
Gastrointestinal bleeding has also been reported in some cases of Menetrier disease usually as a result of erosions or ulcers in the stomach lining. Acid secretion by the stomach is often markedly decreased (hypochlorhydria) or absent. Excess mucous secretion in the stomach may also occur.
Some researchers believe that individuals with Menetrier disease have a greater risk of developing gastric cancer than the general population has. However, other researchers do not believe this to be proven with certainty. In some series, there has been an association between Menetrier disease and ulcerative colitis.
The exact cause of Menetrier disease is unknown. There may be multiple causes. In children, some cases of Menetrier disease may be associated with infection with cytomegalovirus (CMV). The bacterium Helicobacter pylori has been implicated in some adults with Menetrier disease. The exact role, if any that these infections play in the development of Menetrier disease is unknown.
Some researchers believe that the large gastric folds that characterize Menetrier disease may result from increased activation of the epidermal growth factor receptor in the stomach by a protein called transforming growth factor-alpha (TGF).
In extremely rare cases, siblings of affected patients have developed Menetrier disease as children suggesting a genetic link in these cases. Researchers believe that, in these cases, Menetrier disease may be inherited as an autosomal dominant trait. Genetic diseases are determined by two sets of genes, one received from the father and one from the mother. Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary for the appearance of the disease. The abnormal gene can be inherited from either parent, or can be the result of a new mutation (gene change) in the affected individual. The risk of passing the abnormal gene from affected parent to offspring is 50% for each pregnancy regardless of the sex of the resulting child.
Menetrier disease affects males slightly more often than females. It most often affects adults in their 50s or older. However, a childhood form of the disorder exists. Because of the confusion in the literature regarding the term Menetrier disease, it is difficult to determine its true frequency in the general population.
Menetrier disease may be suspected in individuals with large gastric folds. Large gastric folds may be diagnosed by a radiologic study after the patient drinks a barium solution or by an endoscopic exam, a procedure in which a thin, flexible tube (endoscope) is inserted through the mouth and used to examine the interior of the stomach and, if necessary, to obtain tissue samples for microscopic study (biopsy). Histopathologic study of affected stomach tissue obtained by biopsy can support a diagnosis of Menetrier disease. Histopathology is the study of microscopic anatomical changes in diseased tissue.
Treatment
Menetrier disease has been treated with anticholergic drugs, acid suppression therapy, and antibiotic therapy directed against H. pylori infection. These therapies have produced inconsistent results. In some cases, a high-protein diet may be recommended to combat protein loss. Albumin transfusions may also be used to replace albumin loss.
Cetuximab (Erbitux) infusions block the epidermal growth factor receptor and have been successful in some patients with Menetrier disease. Cetuximab treatment can improve quality of life, restore gastric acid production and possibly even reduce the thickness of the stomach wall. In a few cases, after cetuximab infusions are discontinued the patient remains well, at least termporarily. Erbitux may cause cardiopulmary arrest, an acne-like rash and low levels of magnesium, and thus patients must be carefully monitored on this treatment.
In severe cases such as those with significant protein loss or a high probability of progression to gastric cancer, partial or total gastrectomy may be necessary. Gastrectomy is the surgical removal of part or all of the stomach.
In children with Menetrier disease linked to cytomegalovirus infection, antiviral medication directed against cytomegalovirus may lead to improvement of symptoms.
Other treatment is symptomatic and supportive.
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: prpl@cc.nih.gov
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/
Contact for additional information about Menetrier disease:
Mark Feldman, MD
William O. Tschumy Jr. Chair, Department of Internal Medicine
IM Residency Program Director
Texas Health Presbyterian Hospital Dallas
214-345-7881
MarkFeldman@texashealth.org
Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.
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.
INTERNET
Feldman M and Jensen PJ. Large gastric folds: Hyperplastic and nonhyperplastic gastropathies. UpToDate. Last updated: Jul 07, 2015. https://www.uptodate.com/contents/large-gastric-folds-hyperplastic-and-nonhyperplastic-gastropathies?source=see_link Accessed July 27, 2017.
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 July 27, 2017.
NORD strives to open new assistance programs as funding allows. If we don’t have a program for you now, please continue to check back with us.
NORD and MedicAlert Foundation have teamed up on a new program to provide protection to rare disease patients in emergency situations.
Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.
Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.
Learn more https://rarediseases.org/patient-assistance-programs/caregiver-respite/