Unfolding the Diagnosis

Posted by Sara Fazio • April 4th, 2014

In our latest Clinical Problem-Solving article, a previously healthy, 25-year-old man was admitted to the hospital because of abdominal pain, nausea, vomiting, and weight loss. Fever, chills, and weakness developed 2 weeks before his admission.

In protein loss localizable to the gastrointestinal tract, in most cases, the syndrome of protein-losing enteropathy is associated with diseases localized to the small intestine. However, less commonly, the stomach may be a source of protein loss.

Clinical Pearls

What is the differential diagnosis of large gastric mucosal folds on upper endoscopy?

Large gastric mucosal folds are recognized in a variety of conditions and may reflect mucosal hyperplasia (i.e., an excessive number of mucosal epithelial cells confined to the rugae in the gastric body and fundus) or mucosal hypertrophy. As these conditions have the same gross appearance, biopsy is often necessary to determine the cause. Large nonhyperplastic gastric folds may be caused by conditions including chronic H. pylori gastritis, neoplasms such as lymphoma, adenocarcinoma, and carcinoid, as well as lymphocytic gastritis, sarcoidosis, eosinophilic gastroenteritis, and the Cronkhite-Canada syndrome. Hyperplastic gastropathies include the Zollinger-Ellison syndrome, in which there is an increased number of parietal cells, and Menetrier’s disease, in which the number of surface and foveolar mucous cells is increased.

What is the pathogenesis, epidemiology, and clinical presentation of Menetrier’s disease?

The pathogenesis of Menetrier’s disease is incompletely understood. However, the observation of elevated levels of transforming growth factor alpha (TGF-alpha) in gastric mucous cells of patients with this condition has suggested a role for TGF-alpha-induced hyperplasia. Menetrier’s disease affects men more often than women and is most common between ages 40 and 55 years. Clinical manifestations of Menetrier’s disease include epigastric pain, weight loss, nausea, vomiting, gastrointestinal bleeding, diarrhea, and protein-losing gastropathy. Hypoalbuminemia is present in 20 to 100% of patients, according to different series; this feature helps distinguish Menetrier’s disease from the Zollinger-Ellison syndrome.

Morning Report Questions

Q: How is Menetrier’s disease diagnosed and treated?

A: The diagnosis of Menetrier’s disease is usually established by demonstrating extreme foveolar hyperplasia with glandular atrophy and decreased parietal cells on biopsy in a patient with large gastric folds on endoscopy. Treatment includes medications to relieve nausea and gastric pain. A high-protein diet is prescribed to offset the loss of protein. Partial or total gastrectomy is sometimes advocated for patients with intractable pain, hypoalbuminemia, edema, hemorrhage, or pyloric obstruction, or in cases in which malignancy cannot be ruled out. A small single-blinded trial of patients with severe Menetrier’s disease treated for 1 month with cetuximab (a monoclonal antibody that blocks epidermal growth factor receptor signaling) showed significant improvement in quality of life and in biochemical indexes (increased parietal-cell mass and gastric acidity); however, more data are needed to inform the role of this medication in treatment.

Q: What infections have been associated with a Menetrier’s disease-like condition?

A: A Menetrier’s disease-like condition with transient protein-losing gastropathy, hypoalbuminemia, and marked gastric rugal hypertrophy has been reported in association with CMV [cytomegalovirus] infection, mainly in pediatric patients. Menetrier’s disease has also been reported in association with H. pylori. CMV-associated Menetrier’s disease differs from classic Menetrier’s disease in its acute self-limited course and more focal involvement of the gastric fundus, antrum, or both.

9 Responses to “Unfolding the Diagnosis”

  1. julio antonio aljure says:

    EXCELENTE CASO.

  2. nasser says:

    The medical situation is explained very well

  3. saw aung says:

    Very informative. Tq. Wighout tgf alpha , cannot easily be distinguishable from H.pylori infection.

  4. dr nihar says:

    nice one.

  5. Nageen Murad says:

    menetrier s disease a very rare entity, only 200 cases reported. we had one young gentleman 25yrs old, who presented with massive UGI bleed and on EGD after stabilization,he had giant gastric folds sparing antrum. multiple bx were taken. h. pylori was positive. He received eradication therapy which failed despite adequate compliance. further imaging showed para aortic lymph adenopathy with hepatomegaly. multiple bx from stomach suggested menetriers disease. keeping in view of rarity of this disease, full thickness stomach bx was taken and that too suggested menetrier disease and negative for lymphoma IHC stain. main ddx was lymphoma, and on patient desire, he was referred to another physician for further management.

    MENETRIER disease is epithelial (foveolar) hyperplasia and parietal and chief cells replacement by cystically dilated mucus cells. may resolve spontaneously, esp in children where cause is most likely CMV.

  6. Rosa salguero. says:

    Gracias por la informacion ha sido de gran ayuda. Bendiciones.

  7. Paula Campos says:

    Claro y muy útil. Gracias

  8. Petro says:

    Menetiere’s

  9. Kapadiya Maulik says:

    Too nice site to improve the knowledge

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