Ménétrier's disease

Synonyms: Menetriere's disease, giant hypertrophic gastritis, protein-losing gastropathy, hypertrophic gastropathy

What is Ménétrier's disease?¹

This much misspelled disease was named for Pierre Eugène Ménétrier, a French physician (1859-1935) who first described it in 1888.²

Ménétrier’s disease is a rare protein-losing hypertrophic gastropathy. It is characterised by giant gastric rugal folds in the body and fundus, often with antral sparing, decreased acid secretion, increased gastric mucus production, and hypoalbuminaemia secondary to protein loss in the gastric mucosa.

Ménétrier’s disease affects men more frequently than women, and the typical age at diagnosis is between 30 to 60 years.

Ätiologie¹

The pathogenesis of Ménétrier’s disease is not fully understood. It appears that the epidermal growth factor receptor (EGFR) ligand, transforming growth factor alpha, contributes to the pathogenesis.

Ménétrier's disease usually shows an insidious onset with progressive features and is associated with an increased risk of gastric cancer. Variants with abrupt onset and spontaneous remission have been reported. These variants are associated with cytomegalovirus (CMV) or Helicobacter pylori infection. CMV-associated cases usually occur in children, but cases in adults have also been reported.

Ménétrier's disease has also been reported in patients with autoimmune diseases such as inflammatory bowel disease, sclerosing cholangitis, and ankylosing spondylitis, suggesting an immunological component to its pathogenesis.

Ménétrier's disease symptoms (presentation)

Typical clinical presentations include nausea, vomiting, diarrhoea, abdominal pain, weight loss, malnutrition, and peripheral oedema due to hypoalbuminaemia.¹

Differentialdiagnose

The condition needs to be differentiated from other forms of hypertrophic gastropathy such as Zollinger-Ellison syndrome, hypertrophic hypersecretory gastropathy, lymphoma, amyloidosis, lymphocytic gastritis, or gastric carcinoma. ¹

Nachforschungen¹

The diagnosis of choice is gastroscopy with gastric mucosal biopsy. Endoscopically, the markedly thickened gastric mucosal folds resemble cerebral convolutions and primarily affect the body and fundus but spare the antrum. Gastric pH is increased due to loss of parietal cells, and copious thick mucus production is seen secondary to foveolar hyperplasia.

Spontaneous remission variants associated with CMV or Helicobacter pylori infection should be excluded. CMV-associated Ménétrier's disease usually resolves within several weeks to months. H. pylori eradication can be attempted if the organism is detected.

Other investigations will depend on the differential diagnosis and may include contrast CT scan of the abdomen.³

Ménétrier's disease treatment and management¹

There is currently no standard medical treatment. Reports on efficacy of steroids, anticholinergics, acid suppression, and octreotide are inconsistent.

Partial or total gastrectomy is reserved for patients with intractable or debilitating disease and for cases with a high risk of developing gastric cancer.

The link to TGF-α and EGFR signalling in the pathogenesis of Ménétrier's disease has been explored using a neutralising monoclonal antibody (cetuximab) against EGFR for 7 patients with Ménétrier's disease, with some evidence of efficacy.

Komplikationen

There is a well-documented link between Ménétrier's disease and gastric carcinoma. Regular surveillance is essential due to the increased risk of developing gastric adenocarcinoma, lymphoma or any other features consistent with malignant transformation.⁴