Giant hypertrophic gastritis (also called ‘Ménétrier’s disease’ or ‘giant hypertrophic gastritis with hypoproteinemia’; in English ‘Ménétrier’s disease’ or ‘giant hypertrophic gastritis’, hence the acronym GHG), is a rare, acquired disease of the stomach, affecting in particular its mucosa

Giant hypertrophic gastritis is characterised by the formation of giant hypertrophic folds localised mainly in the gastric fundus and excessive mucus production leading to a marked loss of protein, as well as poor or absent hydrochloric acid production.

The aetiology is unknown, but this form of gastritis is associated with excessive secretion of transforming growth factor alpha (TGF-α).

It is a precancerous lesion, i.e. it increases the risk of suffering from a malignant tumour (cancer) of the stomach.

Ménétrier disease’ owes its name to the French physician Pierre Eugène Ménétrier who first described it in the late 1800s.

Ménétrier’s disease should not be confused with Ménière’s syndrome, which is an inner ear disorder that causes severe vertigo and tinnitus.

The disease is most common in men between 30 and 50 years of age.

Giant hypertrophic gastritis, the causes of Ménétrier’s disease

The specific cause of Ménétrier’s disease is currently unknown, however some risk factors are known that increase the chances of suffering from it.

The risk factors for Ménétrier disease are:

• cytomegalovirus (CMV) infection in children
• infection with Helicobacter pylori in adults
• increased levels of transforming growth factor alpha (TGF-α), a particular factor produced by various cells, a ligand for the epidermal growth factor receptor, normally expressed by gastric mucosa cells and capable of inhibiting gastric acid secretion.

Symptoms and signs

In some cases, especially in the early stages, the disease is asymptomatic (i.e. it does not give any symptoms) or has such unspecific and nuanced symptoms that it is diagnosed early, often by chance during examinations performed for other reasons.

When the disease becomes symptomatic, there is

• abdominal pain in the upper quadrants (particularly in the epigastric area, see picture above);
• nausea;
• vomiting;
• marked reduction in appetite;
• tendency to oedema;
• weight loss;
• fatigue;
• general malaise.

Mild gastrointestinal bleeding occurs in some patients, which is usually due to superficial mucosal erosions: such bleeding may lead to symptoms and signs of anaemia.

Marked gastrointestinal bleeding is a very rare event.

Between 20% and 100% of patients, depending on the time of presentation at a healthcare facility, develop a gastropathy characterised by serum protein loss accompanied by hypoalbuminemia.

Acid gastric hyposecretion is the norm and results from a tendency for the gastric glands to atrophy.

The symptoms and clinical features of Ménétrier’s disease in children are similar to those found in adults, but the disease in children generally tends to be self-limiting and often follows a respiratory infection.

Diagnosis of giant hypertrophic gastritis

The tools that can be used for diagnosis (and differential diagnosis) are:

• anamnesis (collection of the patient’s symptoms and medical history);
• objective examination (collection of signs, in particular abdominal and thoracic examination);
• examination of venous blood;
• laboratory investigations for Cytomegalovirus and Helicobacter Pylori;
• blood tests for tumour markers such as carcinoembryonic antigen (CEA) and carbohydrate antigen (CA 19-9);
• chest X-ray with contrast medium (barium meal);
• abdominal radiography;
• abdominal ultrasound;
• breath test;
• endoscopic examination (oesophagogastroduodenoscopy);
• biopsy during endoscopic examination with histological analysis of the specimen;
• CT scan (computed tomography);
• PET (positron emission tomography);
• endoscopic ultrasound (echo-endoscopy).

Not all the listed examinations are always necessary. Hypertrophic gastric folds, typical of Ménétrier’s disease, are easily detected by performing a baritone meal or by endoscopic examinations.

Endoscopy of the upper gastrointestinal tract combined with mucosal biopsy (and cytological examination) is necessary to establish the diagnosis and exclude other disorders that may present in the same way.

In the case of a non-diagnostic endoscopic biopsy, it may be necessary to perform a full-thickness surgical biopsy, which is particularly useful for ruling out a neoplasm.

The differential diagnosis arises with diseases and syndromes presenting similar symptoms and signs, including:

• gastritis;
• gastric infections (from cytomegalovirus, histoplasmosis, syphilis…);
• Zollinger-Ellison syndrome;
• Cronkhite-Canada syndrome;
• Sjögren’s syndrome;
• stomach cancer;
• sarcoidosis.

Therapies for giant hypertrophic gastritis

Treatment is broad-spectrum, drugs such as anticholinergics and corticosteroids are administered, but surgical interventions such as vagotomy and pyloroplasty are also used.

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Source:

Medicina Online