Pathogenesis and Clinical Management of Ménétrier's Disease

Ménétrier’s disease is a rare hyperplastic gastropathy characterised by marked enlargement of gastric rugal folds, foveolar hyperplasia and atrophy of the oxyntic glands. Excessive mucous cell proliferation leads to protein-losing gastropathy, hypoalbuminaemia and peripheral oedema. The precise aetiology remains incompletely understood but overexpression of transforming growth factor-alpha (TGF-α) with subsequent activation of the epidermal growth factor receptor (EGFR) pathway is thought to drive mucosal hyperplasia. Associations with Helicobacter pylori infection in adults and cytomegalovirus in paediatric cases suggest that inflammatory stimuli may act as triggers in susceptible individuals. Clinically, patients present with epigastric pain, anorexia, vomiting, weight loss and signs of protein depletion; in some cases, acquired thrombophilia and deep venous thrombosis have been described as secondary phenomena.

Diagnosis relies on correlation of endoscopic appearance—giant cerebriform rugal folds visible on imaging and direct inspection—with histopathological confirmation of foveolar hyperplasia, glandular cystic dilation and glandular atrophy. Management begins with supportive measures including nutritional supplementation, proton-pump inhibition and eradication of coexistent H. pylori. Targeted therapies have been developed to disrupt EGFR signalling, most notably monoclonal antibodies against EGFR, and somatostatin analogues such as octreotide have shown efficacy in reducing protein loss and mucosal proliferation. For patients refractory to medical therapy or those at high risk of malignant transformation, partial or total gastrectomy remains the definitive intervention. Emerging data suggest that integration of novel biologics with traditional surgical and supportive approaches may improve long-term outcomes.

Research from Nature Portfolio

No recent Nature Portfolio content available.

Pathogenesis and Clinical Management of Ménétrier's Disease publication trend

The graph below shows the total number of articles in pathogenesis and clinical management of ménétrier's disease across all publications each year (not limited to Nature Index journals).

Technical terms

Foveolar hyperplasia: Proliferation of surface mucous cells in the gastric epithelium.

Protein-losing gastropathy: Excessive loss of plasma proteins through the gastric mucosa.

Transforming growth factor-alpha (TGF-α): A growth factor implicated in mucosal cell proliferation.

Epidermal growth factor receptor (EGFR): A cell-surface receptor that mediates growth signals in gastric mucosa.

Rugal folds: The longitudinal mucosal folds lining the stomach.

Hypoalbuminaemia: A condition characterised by low albumin concentration in the blood.

References

1. Ménétrier’s disease presenting as recurrent unprovoked venous thrombosis: a case report. Journal of Medical Case Reports (2019).
2. Clinical and Radiological Features of Menetrier’s Disease: A Case Report and Review of the Literature. Cureus (2023).
3. The Great Mimicker of Gastric Cancer: A Case Report of Ménétrier's Disease. Cureus (2024).
4. Total gastrectomy for the treatment of Menetrier’s disease persistent to medical therapy: A case report. International Journal of Surgery Case Reports (2020).
5. Role of Octreotide in Menetrier’s Disease: Case Report and Review of Literature. Cureus (2020).
6. Ménétrier Disease; 2-Year-Old Boy Presenting With Prolonged Emesis, Generalized Edema and Chance Histopathological Finding of Cytomegalovirus Gastritis: A Case Report. Archives of Medical Case Reports and Case Study (2021).