Ménétrier disease

A rare, acquired, premalignant disease of the stomach

Ménétrier disease
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Abdominal pain, nausea, vomiting, edema, weight loss
Complications	Protein-losing enteropathy, gastric cancer
Onset
Duration
Types	N/A
Causes	Unknown, possibly related to cytomegalovirus infection in children
Risks
Diagnosis	Endoscopy, biopsy, imaging studies
Differential diagnosis	N/A
Prevention	N/A
Treatment	Proton pump inhibitors, anticholinergics, octreotide, gastrectomy
Medication	N/A
Prognosis	Variable
Frequency	Rare
Deaths	N/A

Ménétrier disease is a rare, acquired disorder characterized by hypertrophy of the gastric mucosa, leading to large gastric folds, excessive mucus production, and protein loss. It is named after the French physician Pierre Eugène Ménétrier, who first described the condition in 1888.

Pathophysiology

Ménétrier disease involves the overproduction of transforming growth factor alpha (TGF-α), which stimulates the epidermal growth factor receptor (EGFR) pathway, leading to the proliferation of gastric mucosal cells. This results in the thickening of the gastric mucosa and the formation of large, tortuous gastric folds, primarily in the fundus and body of the stomach. The excessive mucus production and protein loss can lead to hypoalbuminemia and edema.

Clinical Presentation

Patients with Ménétrier disease often present with nonspecific gastrointestinal symptoms such as abdominal pain, nausea, and vomiting. Due to protein loss, patients may also experience edema, particularly in the lower extremities, and weight loss. In some cases, patients may develop anemia due to gastrointestinal bleeding.

Diagnosis

The diagnosis of Ménétrier disease is typically made through a combination of clinical evaluation, endoscopy, and biopsy. Endoscopy reveals enlarged gastric folds, while biopsy shows foveolar hyperplasia and glandular atrophy. Imaging studies such as a CT scan or MRI may also be used to assess the extent of gastric involvement.

Treatment

Treatment options for Ménétrier disease are limited and primarily focus on symptom management. Proton pump inhibitors and anticholinergics may be used to reduce gastric acid secretion and alleviate symptoms. Octreotide, a somatostatin analog, has been used to reduce protein loss and improve symptoms in some patients. In severe cases, gastrectomy may be considered.

Prognosis

The prognosis of Ménétrier disease varies. Some patients may experience spontaneous remission, while others may have a chronic course. There is an increased risk of developing gastric cancer, necessitating regular surveillance.

Also see

• Gastritis
• Gastric cancer
• Protein-losing enteropathy
• Hypertrophic gastropathy