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Synonyms: Menetriere's disease, giant hypertrophic gastritis, protein-losing gastropathy, hypertrophic gastropathy
This much-misspelled disease was named for Pierre Eugène Ménétrier, a French physician (1859-1935) who first described it in 1888.
It is a rare condition characterised by gross hypertrophy of the gastric mucosa, resulting in an increased production of mucus, reduced acid secretion and, due to selective serum protein loss across the gastric mucosa, severe hypoproteinaemia. A similar hypertrophic gastritis has been recognised with Helicobacter pylori infection and in children associated with cytomegalovirus infection (a self-limiting condition). Recent work suggests that H. pylori isolated from patients with Ménétrier's disease is genetically different from that isolated from patients with other gastric diseases.
Recent research implicates overproduction of transforming growth factor-alpha with increased signalling of the epidermal growth factor receptor (EGFR) in the pathogenesis of the condition. Activation of the EGFR, a transmembrane receptor with tyrosine kinase activity, triggers a cascade of downstream, intracellular signalling pathways that leads to expansion of the proliferative compartment within the isthmus of the gastric mucosa (oxyntic) cells. The result is that production of gastric mucus is increased and production of gastric acid is decreased. The existence of the disease in twins suggests that at least some cases have a genetic cause.
Symptoms include nausea, epigastric pain, weight loss and diarrhoea ± melaena. The severe hypoproteinaemia can result in ascites and gross oedema.
The condition needs to be differentiated from other forms of hypertrophic gastropathy such as Zollinger-Ellison syndrome, hypertrophic hypersecretory gastropathy, lymphocytic gastritis, or gastric carcinoma.
- The diagnosis of choice is gastroscopy with gastric mucosal biopsy. Characteristic histological changes include foveolar hyperplasia, cystic dilation of pits and reduced numbers of parietal and chief cells. (Foveolar cells are mucus cells that cover the gastric mucosal surface and line the gastric pits. For a diagram of gastric mucosal cells, check this reference.)
- Transabdominal ultrasound can provide additional information which helps to differentiate patients with giant gastric folds associated with malignancy from those with benign conditions.
- There is evidence that Ménétrier's disease is linked to cytomegalovirus in children and H. pylori in adults and, if either of these infections is present, they should be eradicated.
- Chronic disease is sometimes improved with H2 antagonists or proton pump inhibitors.
- Octreotide, a somatostatin analogue, has been used to good effect in some patients and may obviate the need for surgery.
- Therapies targeting increased signalling of the epidermal growth factor receptor (EGFR) have shown promise, including somatostatin analogues and monoclonal antibodies (eg cetuximab) directed against the EGFR. Erbitux®, a monoclonal antibody that blocks ligand binding to EGFR, has shown promising results in some patients.
- Partial gastrectomy is occasionally recommended for persistent symptoms and particularly for uncontrollable protein loss. A total laparoscopic gastrectomy has also been reported.
There is a well documented link between Ménétrier's disease and gastric carcinoma.
Further reading & references
- Definition of Giant hypertrophic gastritis; MedicineNet.com 2004
- Aslam N et al, Protein-Losing Enteropathy, Medscape, Jul 2008
- Stolte M, Batz CH, Bayerdorffer E, et al; Helicobacter pylori eradication in the treatment and differential diagnosis of giant folds in the corpus and fundus of the stomach. Z Gastroenterol. 1995 Apr;33(4):198-201.
- Badov D, Lambert JR, Finlay M, et al; Helicobacter pylori as a pathogenic factor in Menetrier's disease. Am J Gastroenterol. 1998 Oct;93(10):1976-9.
- Toubia N, Schubert ML; Menetrier's Disease. Curr Treat Options Gastroenterol. 2008 Mar;11(2):103-8.
- Ibarrola C, Rodriguez-Pinilla M, Valino C, et al; An unusual expression of hyperplastic gastropathy (Menetrier type) in twins. Eur J Gastroenterol Hepatol. 2003 Apr;15(4):441-5.
- Menetriere's Disease; Menetriere's Disease, Orphanet
- Jain R, Chetty R; Gastric hyperplastic polyps: a review. Dig Dis Sci. 2009 Sep;54(9):1839-46. Epub 2008 Nov 27.
- diSibio G, McPhaul LW, Sarkisian A, et al; Menetrier's disease associated with Kaposi's sarcoma. Exp Mol Pathol. 2008 Dec;85(3):160-4. Epub 2008 Sep 26.
- Histology of the Gastric Gland; Digestion and Nutrition, University of Pittsburgh School of Medicine, 2003
- Okanobu H, Hata J, Haruma K, et al; Giant gastric folds: differential diagnosis at US. Radiology. 2003 Mar;226(3):686-90. Epub 2003 Jan 24.
- Ishikawa T, Ando T, Obayashi H, et al; Helicobacter pylori Isolated from a Patient with Menetrier's Disease Increases Hepatocyte Growth Factor mRNA Expression in Gastric Fibroblasts: Comparison with Helicobacter pylori Isolated from Other Gastric Diseases. Dig Dis Sci. 2007 Nov 8;.
- Ladas SD, Tassios PS, Malamou HC, et al; Omeprazole induces a long-term clinical remission of protein-losing gastropathy of Menetrier's disease. Eur J Gastroenterol Hepatol. 1997 Aug;9(8):811-3.
- Gadour MO, Salman AH, El Samman el Tel W, et al; Menetrier's disease: an excellent response to octreotide. A case report from the Middle East. Trop Gastroenterol. 2005 Jul-Sep;26(3):129-31.
- Settle SH, Washington K, Lind C, et al; Chronic treatment of Menetrier's disease with Erbitux: clinical efficacy and insight into pathophysiology. Clin Gastroenterol Hepatol. 2005 Jul;3(7):654-9.
- Iida F, Sato A, Koike Y, et al; Surgical and pathologic aspects of protein losing gastropathy. Surg Gynecol Obstet. 1978 Jul;147(1):33-7.
- Sanchez C, Brody F, Pucci E, et al; Laparoscopic total gastrectomy for Menetrier's disease. J Laparoendosc Adv Surg Tech A. 2007 Feb;17(1):32-5.
- Hsu CT, Ito M, Kawase Y, et al; Early gastric cancer arising from localized Menetrier's disease. Gastroenterol Jpn. 1991 Apr;26(2):213-7.
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|Original Author: Dr Laurence Knott||Current Version: Dr Laurence Knott|
|Last Checked: 25/08/2010||Document ID: 2448 Version: 24||© EMIS|