Ménétrier's disease: Difference between revisions

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{{SI}}
{{SI}}
{{CMG}}
{{CMG}};{{AE}}{{Akshun}}


{{EH}}
{{SK}} Cystic gastritis; Giant hypertrophic gastritis; Giant mucosal hypertrophy and hyperplastic gastropathy.


==Overview==
==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 (stomach)|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 alpha|TGF-α]]). The altered gastric mucosa [[secretion|secretes]] massive amounts of [[mucus]], resulting in low [[plasma protein]] levels ([[albumin]]) with variable degree of [[inflammation]].


'''Ménétrier's disease''' (also known as ''hyperplastic hypersecretory gastropathy'' and ''giant hypertrophic gastropathy'' and named after a French physician [[Pierre Eugène Ménétrier]], 1859-1935), is a disorder in which the [[stomach|gastric]] [[mucosa|mucosal]] folds ([[rugae]]) are [[Organ hypertrophy|enlarged]]<ref>http://www.gpnotebook.co.uk/cache/-2026569705.htm</ref> (and the total weight of the stomach is increased), making the surface of the stomach look a bit like the [[brain]] [[gyrus|gyri]]. The altered gastric mucosa [[secretion|secretes]] massive amounts of [[mucus]], resulting in low [[plasma protein]] levels. The tissue may be inflamed and may contain ulcers. The disease also causes glands in the stomach to waste away and causes the body to lose fluid containing a protein called [[human serum albumin|albumin]].
There are two forms of the disease: a childhood form and an adult form.  The childhood form has a better prognosis. It affects boys and girls equally, most often after they have a viral illness caused by [[cytomegalovirus]] (CMV) or a bacterial infection caused by ''[[Helicobacter pylori]]''. Children are not born with this disease, and it is not passed from parents to their children.<ref>http://www.madisonsfoundation.org/content/3/1/display.asp?did=413</ref>. The adult form linked with overexpression of [[transforming growth factor alpha]] (TGF-α). The adult variety is four times more common in men, primarily affecting men between ages 30 and 60.
The presenting symptoms are
* pain after the meal (=[[postprandial]]), relieved by [[antacid]]s, is very usual
* [[weight loss]], [[cachexia]]
* peripheral [[edema]], [[ascites]]
* [[anemia]] symptoms secondary to blood loss
Ménétrier's disease increases a person's risk of [[stomach cancer]].<ref>http://www.mamashealth.com/stomach/menetrier.asp</ref>
[[Histology|Microscopically]], the disease is characterized by [[hyperplasia]] of the [[Stomach#Histology of the human stomach|crypts]], which are elongated and may appear cystic or corkscrew-like. Since it predisposes to [[stomach cancer]], periodic [[endoscopy|endoscopic]] surveillance is mandated. CMV-related Ménétrier is often self-limited.
The disease must be diagnosed by x-ray (rare) or endoscopy and biopsy of the stomach.  In adults, treatment may include medications to relieve ulcer symptoms and treat inflammation, and a high-protein diet. Part or all of the stomach may need to be removed if the disease is severe.  Pediatric cases are normally treated for symptoms with the disease clearing up in weeks to months.
Other forms of hyperplastic gastropathy include [[Zollinger-Ellison syndrome]].
==Introduction==
Ménétrier's disease is a rare, 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.
==Historical Perspective==
==Historical Perspective==
In 1888, Pierre Ménétrier, a French pathologist coined the term Ménétrier's disease after describing mucosal hypertrophy involving part or all of the stomach.
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".<ref name="pmid23303980">{{cite journal |vauthors=Coffey RJ, Tanksley J |title=Pierre Ménétrier and his disease |journal=Trans. Am. Clin. Climatol. Assoc. |volume=123 |issue= |pages=126–33; discussion 133–4 |year=2012 |pmid=23303980 |pmc=3540591 |doi= |url=}}</ref>


==Classification==
==Classification==
Line 32: Line 14:


==Pathophysiology==
==Pathophysiology==
The exact pathogenesis of Ménétrier's disease is not fully understood. However, it is thought that Ménétrier's disease is often due to excessive secretion of transforming growth factor alpha (TGF-α).(PMID 18321437) seen especially in infections such as CMV, H.pylori and herpes simplex.
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 alpha|TGF-α)]]. In children, Ménétrier's disease is especially seen with infections such as [[CMV infection|CMV]], [[Helicobacter pylori]] and [[herpes simplex]].<ref name="pmid18321437">{{cite journal |vauthors=Toubia N, Schubert ML |title=Menetrier's Disease |journal=Curr Treat Options Gastroenterol |volume=11 |issue=2 |pages=103–8 |year=2008 |pmid=18321437 |doi= |url=}}</ref><ref name="pmid9775159">{{cite journal |vauthors=Bencharif L, Cathébras P, Bouchou K, Gouilloud S, Fichtner C, Rousset H |title=[Exudative gastroenteropathy revealing primary CMV infection in an immunocompetent adult] |language=French |journal=Rev Med Interne |volume=19 |issue=4 |pages=288–90 |year=1998 |pmid=9775159 |doi= |url=}}</ref><ref name="pmid22748914">{{cite journal |vauthors=Piepoli A, Mazzoccoli G, Panza A, Tirino V, Biscaglia G, Gentile A, Valvano MR, Clemente C, Desiderio V, Papaccio G, Bisceglia M, Andriulli A |title=A unifying working hypothesis for juvenile polyposis syndrome and Ménétrier's disease: specific localization or concomitant occurrence of a separate entity? |journal=Dig Liver Dis |volume=44 |issue=11 |pages=952–6 |year=2012 |pmid=22748914 |doi=10.1016/j.dld.2012.05.019 |url=}}</ref><ref name="pmid8229553">{{cite journal |vauthors=Occena RO, Taylor SF, Robinson CC, Sokol RJ |title=Association of cytomegalovirus with Ménétrier's disease in childhood: report of two new cases with a review of literature |journal=J. Pediatr. Gastroenterol. Nutr. |volume=17 |issue=2 |pages=217–24 |year=1993 |pmid=8229553 |doi= |url=}}</ref><ref name="pmid1773969">{{cite journal |vauthors=Mosnier JF, Flejou JF, Amouyal G, Gayet B, Molas G, Henin D, Potet F |title=Hypertrophic gastropathy with gastric adenocarcinoma: Menetrier's disease and lymphocytic gastritis? |journal=Gut |volume=32 |issue=12 |pages=1565–7 |year=1991 |pmid=1773969 |pmc=1379265 |doi= |url=}}</ref><ref name="pmid2050372">{{cite journal |vauthors=Haot J, Bogomoletz WV, Jouret A, Mainguet P |title=Ménétrier's disease with lymphocytic gastritis: an unusual association with possible pathogenic implications |journal=Hum. Pathol. |volume=22 |issue=4 |pages=379–86 |year=1991 |pmid=2050372 |doi= |url=}}</ref>
*Overproduction of transforming growth factor-α leads to increased signaling of the epidermal growth factor receptor (EGFR), a transmembrane receptor with tyrosine kinase activity.  
*Overproduction of [[Transforming growth factor alpha|transforming growth factor-α]] leads to increased [[Cell signaling|signaling]] of the [[epidermal growth factor receptor]] ([[EGFR]]), a [[transmembrane receptor]] with [[Tyrosine kinase|tyrosine kinase activity.]]
*Upon activation, the EGFR activates a series of intracellular signaling pathways that leads to increased cell proliferation.
*Upon activation, the [[EGFR]] activates a series of [[Intracellular signaling pathway|intracellular signaling pathways]] that leads to increased [[cell proliferation]].
*Excessive secretion of TGF-α may lead to gastric foveolar cell (surface mucous cells) hyperplasia, edema, and variable degrees of inflammation.  
*Excessive [[secretion]] of [[TGF alpha|TGF-α]] may lead to selective expansion of [[gastric]] foveolar cell (surface mucous cell [[hyperplasia]]) with [[edema]], and variable degrees of [[inflammation]]. The [[inflammation]] may lead to superficial erosion of [[gastric mucosa]] or [[ulceration]].
*The normal gastric glands such as chief or parietal cells are replaced by mucus-secreting cells leading to decreased effective gastric acid production.
*[[Hyperplasia]] of [[gastric]] foveolar cells leads to enlarged gastric folds with elongated and tortuous [[gastric pits]]. In addition, there is also excessive [[mucus]] production.
*An increase in intercellular permeability results in protein loss. In this disorder, tight junctions between cells are wider than those found in healthy subjects, and it is believed that proteins traverse the gastric mucosa through these widened spaces.
*Other [[gastric glands]] such as [[Chief cells|chief]] or [[parietal cells]] are replaced by mucus-secreting cells leading to decreased effective [[gastric acid]] production.
*Hyperplasia of gastric foveolar cells leads to excessive mucus production and enlarged gastric folds.
*In Ménétrier's disease, there is widening of [[gap junctions]] and [[tight junctions]] between cells as compared to healthy subjects, and it is believed that [[proteins]] traverse from the [[gastric]] mucosa into the [[gastric]] lumen through these widened spaces. Hence, an increase in [[intracellular]] [[permeability]] results in [[Protein losing enteropathy|protein-losing enteropathy]].
*The gastric pits are elongated and tortuous.
*In a nutshell, mucosal [[hyperplasia]] with decreased number of [[Chief cells|chief]] and [[parietal cells]] leads to excessive [[mucus]] production, [[protein]] loss from the [[stomach]] and subsequent [[hypochlorhydria]] or [[achlorhydria]] However, it may be noted that Ménétrier's disease is typically but not always associated with protein-losing gastropathy and [[hypochlorhydria]].
*In addition, there is widening of gap junctions between gastric epithelial cells which may lead to exudation of protein and protein-losing enteropathy.
*Ménétrier disease has an insidious onset with progressive features and believed to increase the risk of gastric cancer, but the magnitude of this risk is uncertain.
*Mucosal hyperplasia and decreased chief and parietal cells leads to protein loss from the stomach, excessive mucus production, and hypochlorhydria or achlorhydria.
===Genetics===
*However, Ménétrier's disease is typically but not always associated with protein-losing gastropathy and hypochlorhydria.
*[[Genes]] involved in the [[pathogenesis]] of Ménétrier's disease include [[mutation]] in [[SMAD4|SMAD4 gene]] (associated with [[juvenile polyposis]]). [[Mutation]] in [[SMAD4]] gene may lead to a mixed hypertrophic/polypoid gastropathy. A recent study showed that a dominant 1244_1247delACAG [[mutation]] of [[SMAD4]] was identified in each of the subjects with [[juvenile polyposis]] and in Ménétrier's disease.<ref name="pmid27375208">{{cite journal |vauthors=Burmester JK, Bell LN, Cross D, Meyer P, Yale SH |title=A SMAD4 mutation indicative of juvenile polyposis syndrome in a family previously diagnosed with Menetrier's disease |journal=Dig Liver Dis |volume=48 |issue=10 |pages=1255–9 |year=2016 |pmid=27375208 |doi=10.1016/j.dld.2016.06.010 |url=}}</ref>
*Overproduction of [[TGF alpha|TGF-α]] leads to overactivation of [[EGFR]] which may lead to [[hyperplasia]] of surface mucous cells.
*Some researchers suggest a unifying [[hypothesis]] which suggest [[TGF-β]]–[[SMAD4]] pathway inactivation and [[TGF alpha|TGF-α]] [[overexpression]] related to [[Helicobacter pylori]] infection ultimately leads to Ménétrier's disease.<ref name="pmid22748914">{{cite journal |vauthors=Piepoli A, Mazzoccoli G, Panza A, Tirino V, Biscaglia G, Gentile A, Valvano MR, Clemente C, Desiderio V, Papaccio G, Bisceglia M, Andriulli A |title=A unifying working hypothesis for juvenile polyposis syndrome and Ménétrier's disease: specific localization or concomitant occurrence of a separate entity? |journal=Dig Liver Dis |volume=44 |issue=11 |pages=952–6 |year=2012 |pmid=22748914 |doi=10.1016/j.dld.2012.05.019 |url=}}</ref>


===Genetics===
===Associated conditions===
*Genes involved in the pathogenesis of Ménétrier's disease include mutation in SMAD4 associated with juvenile polyposis can lead to a mixed hypertrophic/polypoid gastropathy.
*[[Helicobacter pylori]] infection
** A dominant 1244_1247delACAG mutation of SMAD4 was identified in each of the subjects with JPS as well as in each of the subjects with MD. (PMID 27375208)
*[[CMV]] gastritis
*Overproduction of TGF-α leads to overactivation of EGF which may lead to hyperplasia of surface mucous cells.
*[[Human Immunodeficiency Virus (HIV)|HIV]]
*Some researchers suggest a unifying hypothesis which suggest TGF-β–SMAD4 pathway inactivation and TGF-α overexpression related to Hp infection ultimately leads to Ménétrier's disease(PMID 22748914).
*[[Gastric cancer]]
*[[Pulmonary|Pulmonary infections]]
*[[Thrombotic]] conditions
*[[Sclerosing cholangitis]]
*[[Ankylosing spondylitis]]


===Gross pathology===
===Gross pathology===
*Polypoid gastric folds
*Polypoid and [[hypertrophic]] gastric folds
*Large cobblestone or cerebriform gastric folds  
*Large cobblestone or cerebriform gastric folds  
*In rare cases, Ménétrier's disease may have [[hyperplastic]] gastric polyps


===Microscopic pathology===
===Microscopic pathology===
*Irregular hypertrophic mucosal folds
*Decreased [[Parietal cells|parietal]] and [[chief cells]]
*Mucosa have swollen, spongy appearance subdivided by creases
*[[Intraepithelial lymphocyte|Intraepithelial lymphocytosis]]
*In rare cases, Ménétrier's disease may have hyperplastic gastric polyps
*[[Glandular]] [[tortuosity]] and [[dilation]]
*Decreased parietal and chief cells with replacement by mucous glands.
*Marked reduction in [[Parietal cells|parietal cell]] number with replacement by [[mucous glands]]
*Intraepithelial lymphocytosis
*Glandular tortuosity and dilation,
*Marked reduction in parietal cell number


===Associated conditions===
==Causes==
*H.pylori infection
Ménétrier's disease may be caused by over expression of [[transforming growth factor alpha]] (TGF-α) seen especially with infections such as [[CMV infection|CMV]], [[Helicobacter pylori|H.pylori]] and [[herpes simplex]].<ref name="pmid18321437">{{cite journal |vauthors=Toubia N, Schubert ML |title=Menetrier's Disease |journal=Curr Treat Options Gastroenterol |volume=11 |issue=2 |pages=103–8 |year=2008 |pmid=18321437 |doi= |url=}}</ref>
*CMV gastritis
*HIV
*Gastric cancer


===Similar conditions===
==Differentiating Ménétrier's disease from Other Diseases==
Conditions that may present with enlarged gastric folds other than Ménétrier's disease include Zollinger Ellison syndrome, inflammatory gastritis, gastric adenomas (lymphoma, carcinoma), granulomatous gastritis, gastric varices, and Zollinger Ellison syndrome and hereditary conditions (such as Familial adenomatous polyposis)
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|Familial Adenomatous Polyposis]]).<ref name="pmid26689786">{{cite journal |vauthors=Huh WJ, Coffey RJ, Washington MK |title=Ménétrier's Disease: Its Mimickers and Pathogenesis |journal=J Pathol Transl Med |volume=50 |issue=1 |pages=10–6 |year=2016 |pmid=26689786 |pmc=4734964 |doi=10.4132/jptm.2015.09.15 |url=}}</ref><ref name="pmid19240670">{{cite journal |vauthors=Blackstone MM, Mittal MK |title=The edematous toddler: a case of pediatric Ménétrier disease |journal=Pediatr Emerg Care |volume=24 |issue=10 |pages=682–4 |year=2008 |pmid=19240670 |doi=10.1097/PEC.0b013e3181887e89 |url=}}</ref><ref name="pmid8482445">{{cite journal |vauthors=Wolfsen HC, Carpenter HA, Talley NJ |title=Menetrier's disease: a form of hypertrophic gastropathy or gastritis? |journal=Gastroenterology |volume=104 |issue=5 |pages=1310–9 |year=1993 |pmid=8482445 |doi= |url=}}</ref><ref name="pmid5316105">{{cite journal |vauthors=Tan DT, Stempien SJ, Dagradi AE |title=The clinical spectrum of hypertrophic hypersecretory gastropathy. Report of 50 patients |journal=Gastrointest. Endosc. |volume=18 |issue=2 |pages=69–73 |year=1971 |pmid=5316105 |doi= |url=}}</ref>
<small>
<small>
{| class="wikitable"
{| class="wikitable"
!align="center" style="background: #4479BA; color: #FFFFFF; " |Disease
! rowspan="2" align="center" style="background: #4479BA; color: #FFFFFF; " |Disease
!align="center" style="background: #4479BA; color: #FFFFFF; " |Age of onset
! rowspan="2" align="center" style="background: #4479BA; color: #FFFFFF; " |Age of onset
!align="center" style="background: #4479BA; color: #FFFFFF; " |Risk factors
! rowspan="2" align="center" style="background: #4479BA; color: #FFFFFF; " |Risk factors
!align="center" style="background: #4479BA; color: #FFFFFF; " |Gastric area involved
! rowspan="2" align="center" style="background: #4479BA; color: #FFFFFF; " |Distribution
!align="center" style="background: #4479BA; color: #FFFFFF; " |Type of gastric glands involved
! rowspan="2" align="center" style="background: #4479BA; color: #FFFFFF; " |Gastric area involved
!align="center" style="background: #4479BA; color: #FFFFFF; " |Inflammatory cells
! rowspan="2" align="center" style="background: #4479BA; color: #FFFFFF; " |Type of gastric glands involved
!align="center" style="background: #4479BA; color: #FFFFFF; " |Symptoms
! rowspan="2" align="center" style="background: #4479BA; color: #FFFFFF; " |Inflammatory cells
!align="center" style="background: #4479BA; color: #FFFFFF; " |Progression to malignancy
! colspan="4" align="center" style="background: #4479BA; color: #FFFFFF; " |Symptoms
! rowspan="2" align="center" style="background: #4479BA; color: #FFFFFF; " |Progression to malignancy
|-
|-
|style="background:#DCDCDC;" |Menetrier's Disease
! align="center" style="background: #4479BA; color: #FFFFFF; " |Nausea
! align="center" style="background: #4479BA; color: #FFFFFF; " |Vomitting
! align="center" style="background: #4479BA; color: #FFFFFF; " |Abdominal pain
! align="center" style="background: #4479BA; color: #FFFFFF; " |Other features
|-
| style="background:#DCDCDC;" |Menetrier's Disease
|40-60s
|40-60s
|H.pylori infection
|[[Helicobacter pylori|H.pylori]] infection
CMV gastritis
[[CMV]] [[gastritis]]
|[[Diffuse]]
|Body & fundus
|Body & fundus
|Mucosal cells
|Mucosal cells
|Lymphocytes
|[[Lymphocytes]]
|Nausea, vomiting, abdominal pain and peripheral edema
| +
| +
| +
|[[Peripheral edema]]
| +
| +
|-
|-
|style="background:#DCDCDC;" |Zollinger Ellison syndrome
| style="background:#DCDCDC;" |[[Zollinger-Ellison syndrome]]
|50s
|50s
|MEN 1 syndrome
|[[MEN 1 syndrome]]
|[[Diffuse]]
|Fundus
|Fundus
|Parietal and mucosal cells
|[[Parietal cell|Parietal]] and mucosal cells
|Neutrophils
|[[Neutrophils]]
|Recurrent peptic ulcers at unsual locations
| +
| +
| +
|Recurrent ulcers
| -
| -
|-
|-
|style="background:#DCDCDC;" |Inflammatory & hyperplastic polyps
| style="background:#DCDCDC;" |Inflammatory & hyperplastic polyps
|50s
|50s
|Gastritis and H.pylori
|[[Gastritis]] and [[Helicobacter pylori|H.pylori]]
|Focal
|Antrum
|Antrum
|Mucosal cells
|Mucosal cells
|Neutrophils and lymphocytes
|[[Neutrophils]] and [[lymphocytes]]
|Nausea, vomiting with epigastric or abdominal pain
| +
| +
| +
|Rectal bleeding with [[diarrhea]] or [[constipation]]
| +/-
| +/-
|-
|-
|style="background:#DCDCDC;" |Granulomatous gastritis
| style="background:#DCDCDC;" |Granulomatous [[gastritis]]
|Variable
|Variable
|History of prior surgery
|History of prior surgery
|[[Diffuse]]
|Body
|Body
|Mucosal cells
|Mucosal cells
|Neutrophils and lymphocytes
|[[Neutrophils]] and [[lymphocytes]]
|Nausea, vomiting with epigastric or abdominal pain
| +
| +
| +
|[[Gastric outlet obstruction]]
| -
| -
|-
|-
|style="background:#DCDCDC;" |Familial adenomatous polyposis
| style="background:#DCDCDC;" |[[Familial adenomatous polyposis]]
|50s
|50s
|Mutation in APC gene
|[[Mutation]] in [[APC gene]]
|Variable
|Body & fundus
|Body & fundus
|Parietal cells
|[[Parietal cells]]
|None
|None
|None
| -
| -
| -
|Asymptomatic to bleeding per rectum
| +
| +
|-
|-
|style="background:#DCDCDC;" |Adenomas (gastric)
| style="background:#DCDCDC;" |[[Adenomas]] (gastric)
|60s
|60s
|Chronic gastritis and intestinal metaplasia
|Chronic gastritis and [[intestinal]] [[metaplasia]]
|Variable
|Antrum
|Antrum
|Dysplastic cells
|[[Dysplastic change|Dysplastic]] cells
|Variable
|Variable
|Nausea, vomiting with epigastric or abdominal pain
| +
| +
| +
|Early [[satiety]]
| +
| +
|}
|}
</small>
</small>


===Diagnostic Criteria===
==Epidemiology and Demographics==
*There is no established criteria for the diagnosis of Ménétrier's disease. However, an endoscopic biopsy, chromium-labeled albumin test (GI protein loss), and 24-hour pH monitoring can establish the diagnosis of Ménétrier's disease. Endoscopy shows typical gastric mucosal changes and biopsy establishes the histopathological variant of  Ménétrier's disease and also to rule out gastric carcinoma or lymphoma.
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.<ref>http://www.madisonsfoundation.org/content/3/1/display.asp?did=413</ref><ref name="pmid3514352">{{cite journal |vauthors=Baker A, Volberg F, Sumner T, Moran R |title=Childhood Menetrier's disease: four new cases and discussion of the literature |journal=Gastrointest Radiol |volume=11 |issue=2 |pages=131–4 |year=1986 |pmid=3514352 |doi= |url=}}</ref><ref name="pmid11519296">{{cite journal |vauthors=Gandhi M, Nagashree S, Murthy V, Hegde R, Viswanath D |title=Menetrier's disease |journal=Indian J Pediatr |volume=68 |issue=7 |pages=685–6 |year=2001 |pmid=11519296 |doi= |url=}}</ref>
*The most accurate method to diagnose Ménétrier's disease includes testing such as oesophagogastroduodenoscopy with gastric pH, serum albumin and full-thickness mucosal biopsy of the involved gastric mucosa.
 
===History and Symptoms===
*Common symptoms of Ménétrier's disease include epigastric pain, dyspepsia, anorexia, vomiting, weight loss, and edema.
*Younger age of onset, male predominance
 
===Prognosis===
The mean age at diagnosis is 5 yr (range: 2 days-17 yr)
Ménétrier's disease usually lasts 2-14 wk, with complete resolution being the rule
 
===Physical Examination===
Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].
 
OR
 
Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
The presence of [finding(s)] on physical examination is diagnostic of [disease name].
 
OR
 
The presence of [finding(s)] on physical examination is highly suggestive of [disease name].
 
===Laboratory Findings===
An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].
 
OR
 
Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
 
OR
 
[Test] is usually normal among patients with [disease name].
 
OR
 
Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].
 
OR
 
There are no diagnostic laboratory findings associated with [disease name].
 
===Electrocardiogram===
There are no ECG findings associated with [disease name].
 
OR
 
An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
===X-ray===
There are no x-ray findings associated with [disease name].
The upper GI series might show thickened gastric folds.
 
OR
 
An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
 
===Echocardiography or Ultrasound===
There are no echocardiography/ultrasound  findings associated with [disease name].
 
OR
 
Echocardiography/ultrasound  may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no echocardiography/ultrasound  findings associated with [disease name]. However, an echocardiography/ultrasound  may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
 
===CT scan===
There are no CT scan findings associated with [disease name].
 
OR
 
[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
 
===MRI===
There are no MRI findings associated with [disease name].
 
OR
 
[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===Other Imaging Findings===
==Risk Factors==
There are no other imaging findings associated with [disease name].
Common risk factors in the development of Ménétrier's disease includes overstimulation of gastric mucosa by [[pituitary]], [[hypothalamic]], or [[vagal]] stimuli.


OR
==Diagnosis==
*There is no established criteria for the diagnosis of Ménétrier's disease. However, an [[endoscopic]] [[biopsy]], chromium-labeled [[albumin]] test (GI protein loss), and 24-hour [[pH]] monitoring can establish the diagnosis of Ménétrier's disease. [[Endoscopy]] with [[biopsy]] can identify the accurate [[histopathology]] and depict the typical gastric mucosal changes of Ménétrier's disease. [[Biopsy]] is also necessary to rule out [[gastric carcinoma]] or [[Gastric lymphoma|lymphoma]].<ref name="pmid20926644">{{cite journal |vauthors=Rich A, Toro TZ, Tanksley J, Fiske WH, Lind CD, Ayers GD, Piessevaux H, Washington MK, Coffey RJ |title=Distinguishing Ménétrier's disease from its mimics |journal=Gut |volume=59 |issue=12 |pages=1617–24 |year=2010 |pmid=20926644 |pmc=3020399 |doi=10.1136/gut.2010.220061 |url=}}</ref>
*The most accurate method to diagnose Ménétrier's disease includes testing such as [[esophagogastroduodenoscopy]] with gastric [[pH]], [[serum albumin]] and full-thickness [[mucosal]] biopsy of the involved [[gastric mucosa]].


[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
==History and Symptoms==
Common symptoms of Ménétrier's disease include [[epigastric pain]] after meals, [[dyspepsia]], [[nausea and vomiting]], [[anorexia]], [[weight loss]], and [[edema]].


===Other Diagnostic Studies===
==Prognosis==
There are no other diagnostic studies associated with [disease name].
In children, Ménétrier's disease is usually from [[CMV infection|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 [[endoscopy|endoscopic]] surveillance is necessary. <ref>http://www.mamashealth.com/stomach/menetrier.asp</ref>


OR
==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.


[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
==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.<ref name="pmid20926644">{{cite journal |vauthors=Rich A, Toro TZ, Tanksley J, Fiske WH, Lind CD, Ayers GD, Piessevaux H, Washington MK, Coffey RJ |title=Distinguishing Ménétrier's disease from its mimics |journal=Gut |volume=59 |issue=12 |pages=1617–24 |year=2010 |pmid=20926644 |pmc=3020399 |doi=10.1136/gut.2010.220061 |url=}}</ref>


OR
==X-ray==
A [[barium meal]] may be helpful in the [[diagnosis]] of Ménétrier's disease. Findings on an [[Upper gastrointestinal series|upper GI series]] suggestive of Ménétrier's disease include thickened and lobulated gastric folds located in gastric [[Fundus (stomach)|fundus]] and body.


Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].
==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]].


===Medical Therapy===
==Other Imaging Findings==
Tthe 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.
[[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.


===Surgery===
==Medical Therapy==
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.
The mainstay of therapy for Ménétrier's disease is supportive care with intravenous [[albumin]] (if symptomatic) and [[Parenteral nutrition|parenteral nutritional]] supplementation. However, some benefit has also been observed with the use of [[anticholinergic]] drugs, acid suppression, [[octreotide]] and [[Helicobacter pylori|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==
==References==
{{reflist|2}}


<references />


==Additional Resources==
==Additional Resources==

Latest revision as of 20:55, 26 January 2018

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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 3540591. 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. doi:10.1016/j.dld.2012.05.019. PMID 22748914.
  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 1379265. 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. doi:10.1016/j.dld.2016.06.010. PMID 27375208.
  9. Huh WJ, Coffey RJ, Washington MK (2016). "Ménétrier's Disease: Its Mimickers and Pathogenesis". J Pathol Transl Med. 50 (1): 10–6. doi:10.4132/jptm.2015.09.15. PMC 4734964. PMID 26689786.
  10. Blackstone MM, Mittal MK (2008). "The edematous toddler: a case of pediatric Ménétrier disease". Pediatr Emerg Care. 24 (10): 682–4. doi:10.1097/PEC.0b013e3181887e89. PMID 19240670.
  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. doi:10.1136/gut.2010.220061. PMC 3020399. PMID 20926644.
  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

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