Mntrier's disease - Wikipedia, the free encyclopedia

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Mntrier's disease
From Wikipedia, the free encyclopedia

Mntrier disease (also known as

hypoproteinemic hypertrophic
gastropathy; named after a French
physician Pierre Eugne Mntrier,
18591935), is a rare, acquired,
premalignant disease of the stomach
characterized by massive gastric
folds, excessive mucous production
with resultant protein loss, and little
or no acid production. The disorder is
associated with excessive secretion of
transforming growth factor alpha
(TGF-).[1]

Mntrier disease

Gross specimen of biopsy of stomach in Mntrier disease. In this case, the

substantial pit hyperplasia makes the large rugal folds appear to be covered by
myriad polyps resembling hyperplastic polyps. The muscularis propria is the
folded structure at the bottom center.
Classification and external resources

Contents
ICD-10

1 Signs and symptoms

2 Cause
3 Pathology
4 Diagnosis
5 Treatment
6 References

K29.6
(http://apps.who.int/classifications/icd10/browse/2015/en#/K29.6)

ICD-9-CM 535.2 (http://www.icd9data.com/getICD9Code.ashx?icd9=535.2)

OMIM

137280 (http://omim.org/entry/137280)

DiseasesDB 8001 (http://www.diseasesdatabase.com/ddb8001.htm)

MeSH

D005758 (https://www.nlm.nih.gov/cgi/mesh/2015/MB_cgi?
field=uid&term=D005758)

Signs and symptoms

The mean age when signs and symptoms first appear is 30 to 60 years. Individuals with the disease present with
upper abdominal pain, at times accompanied by nausea, vomiting, loss of appetite, and weight loss. Occult
gastrointestinal bleed may occur, but overt bleeding is unusual and, when present, is due to superficial mucosal
erosion. Twenty to 100% of patients, depending on time of presentation, develop a protein-losing gastropathy
accompanied by low blood albumin and edema.[2]
Symptoms and pathological features of Mntrier disease in children are similar to those in adults, but disease
in children is usually self-limited and often follows respiratory infection.[3]
The risk of gastric adenocarcinoma is increased in adults with Mntrier disease.[3][4]

Cause
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The cause of Mntrier disease is unknown, but it has been associated with CMV infection in children and H.
pylori infections in adults.[4] Additionally, increased TGF- has been noted in the gastric mucosa of patients
with the disease.[1]

Pathology
The stomach is characterized by large, tortuous gastric folds in the fundus and body of the stomach, with antrum
generally spared, giving the mucosa a cobblestone or cerebriform (brain-like) appearance.[4] Histologically, the
most characteristic feature is massive foveolar hyperplasia (hyperplasia of surface and glandular mucous
cells).[2] The glands are elongated with a corkscrew-like appearance and cystic dilation is common.
Inflammation is usually only modest, although some cases show marked intraepithelial lymphocytosis. Diffuse
or patchy glandular atrophy, evident as hypoplasia of parietal and chief cells, is typical.[3]
Although ICD-10 classifies it under "Other gastritis" (K29.6
(http://apps.who.int/classifications/icd10/browse/2010/en#/K29.6)), and the lamina propria may contain mild
chronic inflammatory infiltrate, Mntrier disease is not considered a form of gastritis.[2] It is rather considered
as one of the two most well understood hypertrophic gastropathies; the other being Zollinger-Ellison
syndrome.[3]

Diagnosis
The differential diagnosis of large gastric folds includes Zollinger-Ellison syndrome, cancer, infectious
etiologies (CMV, histoplasmosis, syphilis), and infiltrative disorders such as sarcoidosis.[2]
Large gastric folds are readily detectable by either radiographic (barium meal) or endoscopic methods.
Endoscopy with deep mucosal biopsy (and cytology) is required to establish the diagnosis and exclude other
entities that may present similarly. A nondiagnostic biopsy may lead to a surgically obtained full-thickness
biopsy to exclude malignancy.[2]
Twenty-four-hour pH monitoring reveals hypochlorhydria or achlorhydria, and a chromium-labelled albumin
test reveals increased GI protein loss.[4] Serum gastrin levels will be within normal limits.

Treatment
Medical therapy with anticholinergic agents, prostaglandins, proton pump inhibitors, prednisone, and H2
receptor antagonists yields varying results. Anticholinergics decrease protein loss. A high-protein diet should be
recommended to replace protein loss in patients with hypoalbuminemia. Ulcers should be treated with the
standard approach. Severe disease with persistent and substantial protein loss may require total removal of the
stomach. Subtotal gastrectomy is performed by some; it may be associated with higher morbidity and mortality
secondary to the difficulty in obtaining a patent and long-lasting anastomosis between normal and hyperplastic
tissue. In adults, there is no FDA approved treatment other than gastrectomy and a high-protein diet.

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More recently, agents that block TGF--mediated activation

of the epidermal growth factor receptor (EGFR) have shown
promise. Cetuximab, a monoclonal antibody against EGFR,
has been used in compassionate use for treatment of
Mntrier's disease.[5]
Pediatric cases are normally treated for symptoms with the
disease clearing up in weeks to months.

References
CT abdomen, coronal section, showing
1. Coffey RJ et al.: Menetrier disease and gastrointestinal stromal
tumors: hyperproliferative disorders of the stomach
characteristic large rugal folds in the stomach. A
(http://www.jci.org/articles/view/30491). J Clin Invest.
cyst is also seen in the liver.
2007;117(1):7080. doi:10.1172/JCI30491
2. Harrison's Principle of Internal Medicine, 18e, pg 2459
3. Robbins and Cotran, Pathological Basis of Disease, 8e, pg 782
4. Townsend et al., Sabiston Textbook of Surgery, 18e, pg 1272
5. Burdick JS, Chung E, Tanner G et al. (December 2000). "Treatment of Mntrier's disease with a monoclonal antibody
against the epidermal growth factor receptor" (http://www.nejm.org/doi/full/10.1056/NEJM200012073432305). N. Engl.
J. Med. 343 (23): 1697701. doi:10.1056/NEJM200012073432305
(https://dx.doi.org/10.1056%2FNEJM200012073432305). PMID 11106719
(https://www.ncbi.nlm.nih.gov/pubmed/11106719).

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Categories: Stomach disorders Hypertrophic gastropathies
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