Ménétrier's disease

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Akshun Kalia M.B.B.S.[2]

Synonyms and Keywords: Cystic gastritis; Giant hypertrophic gastritis; Giant mucosal hypertrophy and hyperplastic gastropathy.

Overview

Ménétrier's disease is a rare, idiopathic, premalignant disease of the mucous glands of the stomach characterized by massive gastric folds. The most commonly involved location is the fundus and corpus of the stomach. It is thought that Ménétrier's disease is often due to excessive secretion of transforming growth factor alpha (TGF-α). The altered gastric mucosa secretes massive amounts of mucus, resulting in low plasma protein levels (albumin) with variable degree of inflammation.

Historical Perspective

In 1888, Pierre Ménétrier, a French pathologist coined the term Ménétrier's disease after describing patients with mucosal hypertrophy involving part or all of the stomach in his journal "Archives Physiologie Normale et Pathologique".[1]

Classification

There is no established system for the classification of Ménétrier's disease.

Pathophysiology

The exact pathogenesis of Ménétrier's disease is not fully understood. However, it is thought that Ménétrier's disease (in children & adults) is often due to excessive secretion of transforming growth factor alpha (TGF-α). In children, Ménétrier's disease is especially seen with infections such as CMV, Helicobacter pylori and herpes simplex.[2][3][4][5][6][7]

Genetics

Associated conditions

Gross pathology

  • Polypoid and hypertrophic gastric folds
  • Large cobblestone or cerebriform gastric folds
  • In rare cases, Ménétrier's disease may have hyperplastic gastric polyps

Microscopic pathology

Causes

Ménétrier's disease may be caused by over expression of transforming growth factor alpha (TGF-α) seen especially with infections such as CMV, H.pylori and herpes simplex.[2]

Differentiating Ménétrier's disease from Other Diseases

Ménétrier's disease must be differentiated from other conditions that presents with enlarged/massive gastric folds such as Zollinger-Ellison syndrome, inflammatory gastritis, granulomatous gastritis, gastric adenomas (lymphoma/carcinoma), and hereditary conditions (such as Familial Adenomatous Polyposis).[9][10][11][12]

Disease Age of onset Risk factors Distribution Gastric area involved Type of gastric glands involved Inflammatory cells Symptoms Progression to malignancy
Nausea Vomitting Abdominal pain Other features
Menetrier's Disease 40-60s H.pylori infection

CMV gastritis

Diffuse Body & fundus Mucosal cells Lymphocytes + + + Peripheral edema +
Zollinger-Ellison syndrome 50s MEN 1 syndrome Diffuse Fundus Parietal and mucosal cells Neutrophils + + + Recurrent ulcers -
Inflammatory & hyperplastic polyps 50s Gastritis and H.pylori Focal Antrum Mucosal cells Neutrophils and lymphocytes + + + Rectal bleeding with diarrhea or constipation +/-
Granulomatous gastritis Variable History of prior surgery Diffuse Body Mucosal cells Neutrophils and lymphocytes + + + Gastric outlet obstruction -
Familial adenomatous polyposis 50s Mutation in APC gene Variable Body & fundus Parietal cells None - - - Asymptomatic to bleeding per rectum +
Adenomas (gastric) 60s Chronic gastritis and intestinal metaplasia Variable Antrum Dysplastic cells Variable + + + Early satiety +

Epidemiology and Demographics

Ménétrier's disease is seen in both children and adults. In children, Ménétrier's disease affects boys and girls equally. In adults, Ménétrier's disease is more commonly seen in the age group of 30 to 50 years with men four times more commonly affected than women.[13][14][15]

Risk Factors

Common risk factors in the development of Ménétrier's disease includes overstimulation of gastric mucosa by pituitary, hypothalamic, or vagal stimuli.

Diagnosis

History and Symptoms

Common symptoms of Ménétrier's disease include epigastric pain after meals, dyspepsia, nausea and vomiting, anorexia, weight loss, and edema.

Prognosis

In children, Ménétrier's disease is usually from CMV infection and lasts for 2-14 weeks, with complete resolution being the rule. In adults, untreated Ménétrier's disease may increase a patient's risk of stomach cancer. Thus, a periodic endoscopic surveillance is necessary. [17]

Physical Examination

Patients with Ménétrier's disease usually appear fatigued and cachexic. Common physical examination findings of patients with Ménétrier's disease includes abdominal tenderness, peripheral edema and with signs of iron deficiency anemia (secondary to blood loss from ulcers) such as pallor and brittle nails. Patients with prolonged Ménétrier's disease may also have ascites and abdominal distension from protein loss.

Laboratory Findings

Laboratory findings consistent with the suggestive of Ménétrier's disease include hypoalbuminemia and hypochlorhydria resulting from protein-losing enteropathy and decreased acid secretion respectively. Other tests include complete blood count and metabolic panel to determine the severity of the disease.[16]

X-ray

A barium meal may be helpful in the diagnosis of Ménétrier's disease. Findings on an upper GI series suggestive of Ménétrier's disease include thickened and lobulated gastric folds located in gastric fundus and body.

CT scan

Abdominal CT scan may be helpful in the diagnosis of Ménétrier's disease. Findings on CT scan suggestive of Ménétrier's disease include massive lobulated gastric folds. An important feature of Ménétrier's disease which differentiates it from gastric carcinoma includes the presence of pliable gastric folds as compared to rigid and aperistaltic gastric folds seen in carcinoma.

Other Imaging Findings

Endoscopy may be helpful in the diagnosis of Ménétrier's disease. Findings on an endoscopy suggestive of Ménétrier's disease include enlarged, nodular and coarse gastric folds.

Medical Therapy

The mainstay of therapy for Ménétrier's disease is supportive care with intravenous albumin (if symptomatic) and parenteral nutritional supplementation. However, some benefit has also been observed with the use of anticholinergic drugs, acid suppression, octreotide and H. pylori eradication.

Surgery

Surgery is not the first-line treatment option for patients with Ménétrier's disease. Surgery (subtotal/total gastrectomy) is usually reserved for patients with either massive protein loss unresponsive to medical therapy or with dysplasia or carcinoma.

References

  1. Coffey RJ, Tanksley J (2012). "Pierre Ménétrier and his disease". Trans. Am. Clin. Climatol. Assoc. 123: 126–33; discussion 133–4. PMC 3540591Freely accessible. PMID 23303980. 
  2. 2.0 2.1 Toubia N, Schubert ML (2008). "Menetrier's Disease". Curr Treat Options Gastroenterol. 11 (2): 103–8. PMID 18321437. 
  3. Bencharif L, Cathébras P, Bouchou K, Gouilloud S, Fichtner C, Rousset H (1998). "[Exudative gastroenteropathy revealing primary CMV infection in an immunocompetent adult]". Rev Med Interne (in French). 19 (4): 288–90. PMID 9775159. 
  4. 4.0 4.1 Piepoli A, Mazzoccoli G, Panza A, Tirino V, Biscaglia G, Gentile A, Valvano MR, Clemente C, Desiderio V, Papaccio G, Bisceglia M, Andriulli A (2012). "A unifying working hypothesis for juvenile polyposis syndrome and Ménétrier's disease: specific localization or concomitant occurrence of a separate entity?". Dig Liver Dis. 44 (11): 952–6. PMID 22748914. doi:10.1016/j.dld.2012.05.019. 
  5. Occena RO, Taylor SF, Robinson CC, Sokol RJ (1993). "Association of cytomegalovirus with Ménétrier's disease in childhood: report of two new cases with a review of literature". J. Pediatr. Gastroenterol. Nutr. 17 (2): 217–24. PMID 8229553. 
  6. Mosnier JF, Flejou JF, Amouyal G, Gayet B, Molas G, Henin D, Potet F (1991). "Hypertrophic gastropathy with gastric adenocarcinoma: Menetrier's disease and lymphocytic gastritis?". Gut. 32 (12): 1565–7. PMC 1379265Freely accessible. PMID 1773969. 
  7. Haot J, Bogomoletz WV, Jouret A, Mainguet P (1991). "Ménétrier's disease with lymphocytic gastritis: an unusual association with possible pathogenic implications". Hum. Pathol. 22 (4): 379–86. PMID 2050372. 
  8. Burmester JK, Bell LN, Cross D, Meyer P, Yale SH (2016). "A SMAD4 mutation indicative of juvenile polyposis syndrome in a family previously diagnosed with Menetrier's disease". Dig Liver Dis. 48 (10): 1255–9. PMID 27375208. doi:10.1016/j.dld.2016.06.010. 
  9. Huh WJ, Coffey RJ, Washington MK (2016). "Ménétrier's Disease: Its Mimickers and Pathogenesis". J Pathol Transl Med. 50 (1): 10–6. PMC 4734964Freely accessible. PMID 26689786. doi:10.4132/jptm.2015.09.15. 
  10. Blackstone MM, Mittal MK (2008). "The edematous toddler: a case of pediatric Ménétrier disease". Pediatr Emerg Care. 24 (10): 682–4. PMID 19240670. doi:10.1097/PEC.0b013e3181887e89. 
  11. Wolfsen HC, Carpenter HA, Talley NJ (1993). "Menetrier's disease: a form of hypertrophic gastropathy or gastritis?". Gastroenterology. 104 (5): 1310–9. PMID 8482445. 
  12. Tan DT, Stempien SJ, Dagradi AE (1971). "The clinical spectrum of hypertrophic hypersecretory gastropathy. Report of 50 patients". Gastrointest. Endosc. 18 (2): 69–73. PMID 5316105. 
  13. http://www.madisonsfoundation.org/content/3/1/display.asp?did=413
  14. Baker A, Volberg F, Sumner T, Moran R (1986). "Childhood Menetrier's disease: four new cases and discussion of the literature". Gastrointest Radiol. 11 (2): 131–4. PMID 3514352. 
  15. Gandhi M, Nagashree S, Murthy V, Hegde R, Viswanath D (2001). "Menetrier's disease". Indian J Pediatr. 68 (7): 685–6. PMID 11519296. 
  16. 16.0 16.1 Rich A, Toro TZ, Tanksley J, Fiske WH, Lind CD, Ayers GD, Piessevaux H, Washington MK, Coffey RJ (2010). "Distinguishing Ménétrier's disease from its mimics". Gut. 59 (12): 1617–24. PMC 3020399Freely accessible. PMID 20926644. doi:10.1136/gut.2010.220061. 
  17. http://www.mamashealth.com/stomach/menetrier.asp


Additional Resources

  • Rubin's Pathology, Clinicopathological Foundations of Medicine, 4th edition, Rubin, Gorstein, Rubin, Schwarting, Strayer. Lippincott Williams & Wilkins. ISBN 0-7817-4733-3

Cost Effectiveness of Ménétrier's disease

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| group6 = Guidelines / Policies / Government Resources (FDA/CDC) Regarding Ménétrier's disease | list6 = US National Guidelines Clearinghouse on Ménétrier's diseaseNICE Guidance on Ménétrier's diseaseNHS PRODIGY GuidanceFDA on Ménétrier's diseaseCDC on Ménétrier's disease


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| group9 = Genetics, Pharmacogenomics, and Proteinomics of Ménétrier's disease | list9 = AND (pharmacogenomics)}} Genetics of Ménétrier's diseaseAND (pharmacogenomics)}} Pharmacogenomics of Ménétrier's diseaseAND (proteomics)}} Proteomics of Ménétrier's disease


| group10 = Newstories on Ménétrier's disease | list10 = Ménétrier's disease in the newsBe alerted to news on Ménétrier's diseaseNews trends on Ménétrier's disease


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| group12 = Patient Resources on Ménétrier's disease | list12 = Patient resources on Ménétrier's diseaseDiscussion groups on Ménétrier's diseasePatient Handouts on Ménétrier's diseaseDirections to Hospitals Treating Ménétrier's diseaseRisk calculators and risk factors for Ménétrier's disease


| group13 = Healthcare Provider Resources on Ménétrier's disease | list13 = Symptoms of Ménétrier's diseaseCauses & Risk Factors for Ménétrier's diseaseDiagnostic studies for Ménétrier's diseaseTreatment of Ménétrier's disease

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| group17 = Informatics Resources on Ménétrier's disease | list17 = List of terms related to Ménétrier's disease


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