Menetrier's Disease: Epidemiology, Etiopathogenesis

Menetrier's Disease

Menetrier’s Disease is also called Hypertrophic and hypoproteinemic(associated with protein loss) disease of the stomach.

Epidemiology Of Menetrier’s Disease

• It can affect any age range, but the majority of the cases are diagnosed in middle age (30-60 yrs)
• It is seen more commonly in the male population than in the female population.

Etiopathogenesis of Mentrier’s disease

• It is an acquired disorder and not an inherited one. The genetic disorder and genetic abnormality associated with Menetrier's disease are not known yet.
• Infection
  • Both H.Pylori and CMV can cause menetrier's disease in a reversible form. The reversible form usually regresses in 6 months,
  • Another is the progressive form of menetrier's disease, which persists for a long time.
• The infection is primarily in the stomach. The Fundus and body of the stomach are affected, whereas the distal part of the stomach, which is the antrum, is spared.

Pathophysiology of Mentrier’s disease

• The pathophysiology of Menetrier disease remains incompletely elucidated. It is believed that heightened signaling through the epidermal growth factor receptor (EGFR), induced By increased production of TGF-alpha, results in the proliferation of mucosal epithelial cells. 
• TGF-alpha, a ligand that activates EGFR, stimulates the growth of these cells, which, in excess, produce copious amounts of mucus. TGF-alpha also contributes to reduced acid production, directly affecting parietal cells and indirectly promoting somatostatin release. 
• Excessive mucus secretion leads to malabsorption of nutrients, electrolytes, and vitamins in the small intestine, culminating in the clinical syndrome of protein-losing gastropathy.

Histopathology of Mentrier’s disease

• Surface mucous cells are also known as foveolar cells.
• Mucous neck cells can regenerate surface mucous cells.
• Both surface mucous cells and mucous neck cells can secrete mucus, but the basic difference is mucus secreted by surface mucous cells is bicarbonate-rich fluid, and mucous neck cells secrete acidic fluid.
• There is foveolar hyperplasia with decreased parietal and chief cell mass.
  • As parietal cells secrete Hcl and Intrinsic Factors, the patient may develop Achlorhydria( absence of hydrochloric acid in the gastric secretions) and increased gastrin secretion.
  • Decreased pepsinogen is also seen because of decreased chief cell mass.
• In foveolar hyperplasia, gastric glands, mainly pits, become elongated and tortuous.

Clinical features of Mentrier’s disease

• Clinical features related to abdominal and gut are:
  • Abdominal pain 
  • Diarrhea
  • Nausea 
  • Vomiting
• Clinical features secondary to hypoalbuminemia are due to the main defect of the malabsorption/ loss of protein that occurs in Menetrier’s disease. These will be mainly anasarca, a very severe condition in which abnormal fluid accumulation between the body cells and the body cavity occurs.

Diagnosis of Mentrier’s disease

Diagnosis can be made by certain techniques:

1. Biopsy:
   1. Tortuosity of glands
   2. Dilatation of glands
   3. Prominent eosinophils and/or plasma cell clusters in lamina propria
   4. Lamina propria smooth muscle hyperplasia & edema
      • If all four of these changes are seen in a patient’s biopsy. Only then 
2. Barium study: This shows markedly enlarged folds along the greater curvature of the stomach. The antrum is spared. Barium is diluted due to excess mucus secretion, which is seen as an impaired mucosal coating. 
3. On contrast-enhanced computed tomography (CECT) images, diffuse gastric mucosal thickening is seen.
4. CT scan 
5. Laboratory evaluation should also be done, which includes a complete blood count, H. pylori & CMV serology, Serum albumin, Serum gastrin.

Differential Diagnosis Of Menetrier’s Disease

• Zollinger-Ellison syndrome (ZES)
• Effect of PPI’s
• Infiltrative disease
• Hypertrophic lymphocytic gastritis
• Gastric adenocarcinoma
• Hypertrophic hypersecretory gastropathy
• H. pylori gastritis
• Polyposis syndrome: The diagnosis of polyposis syndrome can be made when there is
  • Prominent edema
  • Lack of lamina propria 
  • smooth muscle hyperplasia
  • Plasma cells and/or eosinophils may be present in the lamina propria.

Management Of Menetrier’s Disease

• Gastrectomy- this is the surgical resection of the stomach’s area, which has histological changes.
• Cetuximab
  • Replaced gastrectomy as the initial treatment of choice
  • It is a monoclonal antibody against epidermal growth factor receptor (EGFR). It blocks EGFR signaling.

                                        PGF-α 

                                             ↓ (blocked by cetuximab)

                                 Activate EGFR signal  

• This is used as an IV infusion in the patient as this method will give the drug the highest bioavailability.
• 50% of patients are able to achieve remission when treated with cetuximab
• PPIs
• Anticholinergics

Prognosis of Menetrier’s Disease

Menetrier’s Disease is a self-limiting disorder and usually resolves within a few weeks to months. However, it should be treated actively for other diseases that occur with it.

Also Read: Mesenteric Ischemia: Causes, Symptoms, and Treatments

Hope you found this blog helpful for your NEET SS Gastroenterology and Hepatobiliary preparation. For more informative and interesting posts like these, keep reading PrepLadder’s blogs.