Menetrier Disease: causes, symptoms and treatment

Medically reviewed: 4, March 2024

Symptoms: Bloated Stomach

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Menetrier disease pathophysiology

Menetrier Disease, also known as Giant Hypertrophic Gastropathy, is a rare disorder affecting the stomach lining. It results in thickened folds within the stomach walls causing abnormal protein loss, leading to fluid accumulation and swelling. Symptoms may include nausea, vomiting, weight loss, and malnutrition.

Its causes remain uncertain; however, research suggests associations between certain bacterial infections, genetic predisposition, and gastric acid hypersecretion.

Diagnosis involves excluding other conditions manifesting similar symptoms and usually includes endoscopies and biopsies to examine mucous membranes.

Depending on severity, treatments range from nutritional support to medications regulating gastric secretions, antibiotics, antacids, or even surgeries to remove affected portions of the stomach. Consult medical professionals for advice tailored to specific circumstances since information online does not replace expert opinion and personal evaluation.

What Happens Inside Your Body With Menetrier’s Disease?

In normal cases, our digestive system secretes acids to break down food for better absorption. However, when suffering from Menetrier’s Disease, there is overproduction of mucus resulting in inflammation and impaired gastric function. Consequently, nutrients aren’t absorbed correctly, which often leads to complications like diarrhea, indigestion, stomach pain, poor appetite, and unintentional weight loss. In severe instances, patients might develop an increased likelihood of acquiring stomach ulcers and adenocarcinoma, a form of gastric cancer.

Who Is Prone to Getting Menetrier’s Disease?

This condition predominantly affects middle-aged adults, although cases among children have been documented too. Studies suggest no clear racial or ethnic preference towards contracting this illness. Furthermore, researchers believe some individuals inherit genes associated with higher risks of developing Menetrier’s Disease. Still, concrete evidence supporting hereditary patterns remains elusive.

How Do You Know If You Have Menetrier’s Disease?

Diagnosing Menetrier’s Disease isn’t straightforward because many initial symptoms mirror common digestive problems. Typical diagnostic methods include physical examinations supplemented by comprehensive analysis of medical history. Often, doctors rely heavily on investigative procedures involving radiologic imagery, endoscopy, biopsies, and laboratory tests to detect anomalies linked to the disease definitively. Observing characteristic signs, namely fingerlike projections called villi flattening out, confirms the diagnosis.

Treatments and Coping Strategies For Living With Menetrier’s Disease

As of today, treating Menetrier’s Disease relies largely on managing uncomfortable symptoms rather than curing the underlying cause directly. Some remedies involve medication therapy targeting reduced acid production, inflammation suppression, improved nutrition uptake, and combatting potential infection sources. Sometimes, surgery becomes inevitable if polyps turn precancerous or pose significant health threats requiring urgent intervention.

Menetrier Disease: Causes and Development

The exact cause behind Menetrier Disease remains unknown. However, researchers suggest several theories explaining its development. One prominent theory attributes the disease to overproduction of Transforming Growth Factor Alpha (TGF-α). TGF-α plays a significant role in cell growth regulation within our bodies. In some instances, due to genetic predisposition or environmental triggers, the body may produce excess TGF-α leading to uncontrolled proliferation of cells lining the stomach walls causing hypertrophy—enlargement of the rugae or gastric folds.

Another possible explanation relates to viral infections. Certain types of viruses might stimulate an immune response resulting in inflammation and subsequent overgrowth of the stomach linings. Yet another hypothesis suggests bacterial involvement, specifically Helicobacter pylori, commonly associated with peptic ulcers. While evidence connecting H. Pylori directly to Menetrier Disease isn’t conclusive, there appears to be some correlation worth exploring.

Apart from intrinsic factors, extrinsic elements could potentially contribute to the onset of Menetrier Disease too. These can range from long-term use of nonsteroidal anti-inflammatory drugs (NSAIDs), radiation therapy aimed at abdominal areas, and bile reflux — the backflow of bile fluids from the small intestine into the stomach. All these external influences have been observed to irritate the stomach lining and potentially lead to its hyperplasia, though solid proof confirming them as direct causes is still under investigation.

Despite numerous hypotheses regarding the origin of Menetrier Disease, no definitive conclusions exist yet. Most cases seem sporadic without clear precipitating factors. Further research continues to delve deeper into understanding this perplexing condition’s genesis better, aiming towards effective diagnosis, management, and potential cure strategies.

Menetrier Disease: Protein Loss and Associated Complications

Beyond the physical changes occurring in the stomach wall, one major concern surrounding Menetrier Disease involves increased protein excretion, often referred to as hypoproteinemia.

This phenomenon results in insufficient levels of proteins required for vital bodily functions, ultimately giving rise to numerous health complications.

Protein loss primarily happens due to two interconnected mechanisms involved in Menetrier Disease progression. Firstly, the dilated glands present in the stomach mucosa secrete large quantities of mucus rich in proteins. Overwhelmed by this surplus secretion, the damaged stomach epithelium loses the capacity to absorb proteins efficiently, leading to the second mechanism: malabsorption syndrome.

Combined, both circumstances result in substantial protein leakage through the gut, posing severe risks to affected individuals’ overall wellbeing.

Hypoproteinemia entails several hazardous consequences when neglected or improperly managed. Since albumin constitutes approximately half of plasma proteins, declines in serum albumin level serve as a principal indicator of low protein count.

Common ramifications linked to:

  • hypoproteinemia comprise edema (swelling attributed to fluid retention),
  • ascites (abnormal accumulation of fluid within the abdomen),
  • pleural effusion (excess liquid around the lungs),
  • and general weakness due to impaired tissue repair and regeneration brought forth by decreased availability of crucial nutrients and building blocks needed for physiological maintenance.

Moreover, recurrent episodes of diarrhea experienced by patients grappling with Menetrier Disease intensify electrolyte imbalances contributing to hypovolemia – reduced blood volume – thereby hampering proper oxygen distribution throughout the body and impeding optimal organ function.

Children diagnosed with Menetrier Disease face additional challenges owing to compromised growth rates tied to poor nutrition absorption and disruptions in hormonal balance caused by persistent hypoproteinemia.

Managing protein loss incurred by Menetrier Disease necessitates addressing underlying causes triggering exaggerated mucus production alongside introducing dietary modifications supplemented by pharmaceutical interventions.

Nutrient-rich diets fortified with high-quality proteins derived mainly from lean meat, fish, eggs, dairy products, legumes, nuts, and seeds are generally recommended to counteract deficiencies emanating from prolonged protein loss.

Menetrier Disease: Symptoms and Signs

Symptoms of Menetrier Disease can vary significantly among patients. Some may experience mild discomfort, while others suffer severe complications requiring hospitalization. Common indicators include:

  1. Epigastric pain: Discomfort localized in the upper central region of the abdomen, often associated with excessive gas production or indigestion.
  2. Nausea and vomiting: Persistent feelings of queasiness followed by regurgitating food contents or bile, usually worsening after meals.
  3. Weight loss: Unintentional decrease in body weight due to reduced appetite, malabsorption, or protein wasting caused by chronic inflammation.
  4. Abdominal distention: Swelling or bloating in the belly area triggered by fluid accumulation or intestinal gas collection.
  5. Early satiety: Feeling full quickly during or shortly after starting a meal, leading to poor nutritional intake.

Less frequently observed signs encompass iron deficiency anemia, hypoproteinemia, peripheral edema, ascites, and melena (dark stool containing digested blood). These less common symptoms typically result from protein loss across damaged mucosal surfaces and subsequent hematological consequences.

Menetrier Disease Diagnostics

Diagnostic procedures for Menetrier Disease involve several tests aimed at excluding differential diagnoses and assessing disease severity:

  1. Medical history & physical examination: Initial assessment includes reviewing patient complaints, past health records, family history, current medications, lifestyle habits, and performing routine checks on vital organs, reflexes, and overall wellbeing.
  2. Upper endoscopy (EGD): Direct visual inspection of the esophagus, stomach, and duodenum utilizing an endoscope facilitates evaluation of mucosal appearances and detection of potential lesions. Biopsies extracted during EGD confirm histopathological findings characteristic of Menetrier Disease.
  3. Imaging studies: X-ray examinations, CT scans, or MRIs can reveal stomach enlargement, identify structural irregularities, discern masses, or rule out underlying neoplastic conditions mimicking symptomatology.
  4. Laboratory analyses: Complete blood count, serum electrolyte panel, coagulation profile, liver function test, fecal occult blood test, and tumor markers provide additional insights regarding general health status, organ functionality, and possible cancer presence.
  5. Genetic testing: Although no definitive genetic link has been established, certain gene mutations could potentially predispose individuals towards developing Menetrier Disease. Consequently, analyzing DNA sequences might shed light on individual susceptibility or familial inheritance patterns.

Menetrier Disease and Radiology & Endoscopy diagnostics

Imaging Modalities

Various imaging modalities aid in diagnosing Menetrier disease, each presenting distinct advantages depending on clinical circumstances. For instance, Barium Meals have long served as initial investigative tools since their introduction in the late 19th century. However, modern cross-sectional techniques such as Computed Tomography (CT), Magnetic Resonance Imaging (MRI), and Ultrasound offer superior soft tissue resolution, enabling detailed exploration of deeper layers of the gastric wall.

Barium Meals

Historically, double-contrast barium meals were considered the gold standard for diagnosing Menetrier disease due to their ability to highlight characteristic mucosal fold abnormalities. Despite being supplanted by advanced methodologies, conventional radiography retains merits for preliminary assessment, particularly when evaluating symptomatic individuals. Typical radiographic hallmarks include thickened, rigid, nodular, and irregular folds concentrated predominantly in the distal half of the stomach but seldom sparing the cardiac region entirely. Furthermore, intramural pseudodiverticulae formation can result in trapped barium pockets mimicking diverticular disease.

Computed Tomography (CT)

Multidetector row helical CT provides rapid acquisition times combined with thin collimation, enhancing spatial resolution. Resultantly, multiplanar reformatted views facilitate comprehensive investigation of complex anatomical relations. In addition, intravenously administered iodinated contrast agents enable organ perfusion analysis, contributing valuable information towards distinguishing benign conditions from potentially malignant ones.

Classical CT appearances of Menetrier disease show extensive gastric wall thickening exceeding 1cm coupled with prominent mucosal plications manifesting a ‘rugged’ texture. Other common observations entail luminal narrowing attributable to:

  • muscular layer hypertrophy,
  • submucosal edema,
  • fibrosis,
  • or even fatty infiltration.

Incidentally discovered instances warrant thorough workup comprising histopathological confirmation, thus avoiding misinterpretation of incidental findings suggestive of neoplasm.

Magnetic Resonance Imaging (MRI)

MR imaging protocols exploiting heavily T2-weighted sequences permit high-resolution depiction of stomach morphology devoid of ionizing radiation exposure, making them attractive alternatives for pediatric populations and pregnant women.

Recent advances incorporate diffusion-weighted imaging sequences to discern tumorous invaders against background tissues based on varying water molecule motion properties. Nevertheless, routine utilization of MRI in Menetrier disease diagnostics requires rigorous validation given inherent limitations related to longer acquisition periods susceptible to respiratory artifacts, necessitating expert interpretation skills.

Endoscopi and Menetrier Disease

Esophagogastroduodenoscopy (EGD) stands among primary procedures performed following suspicion of Menetrier disease arising from nonspecific symptoms, laboratory derangements, or positive screening results. EGD offers opportunity for acquiring representative specimen suitable for subsequent immunohistochemistry and molecular profiling, thereby guiding therapeutic decision-making.

At endoscopy, Menetrier disease exhibits distinctive macroscopic traits incorporating elevated mucosal folds engulfing expansive gastric territories. Such protuberances often coalesce intermittently creating an undulating landscape traversed by deep furrows likened to a cerebral cortex (‘sulci’) hence termed ‘gyri’. Concurrently, patchy erosions afflict intervening crests inciting secondary bacterial colonization linked to recurrent episodes of superadded acute gastritis. Interestingly, several reports indicate concurrence of peptic ulcerations located chiefly within fundic regions flanking atypically tall folds although underlying mechanisms remain obscure.

Treatment Options for Menetrier Disease: Current Approaches and Future Directions

While there isn’t a single definitive cure for Menetrier Disease, the following treatment approaches can be considered:

  1. Proton Pump Inhibitors (PPIs): These medications work by reducing gastric acid production in the stomach. Long-term PPI therapy may lead to symptomatic relief, improve nutritional status, and diminish protein loss in some patients. Common PPIs include omeprazole, esomeprazole, pantoprazole, rabeprazole, and lansoprazole.
  2. Histamine-2 receptor antagonists (H2 blockers): Similar to PPIs, H2 blockers suppress gastric acid secretion. They might provide temporary relief for milder cases of Menetrier Disease, although typically less effective than PPIs. Examples include ranitidine, famotidine, cimetidine, and nizatidine.
  3. Antibiotics: Short courses of antibiotics have shown benefits in treating certain cases of Menetrier Disease associated with Helicobacter pylori infection. However, evidence regarding their efficacy varies among individual studies, and elimination of H. pylori doesn’t necessarily guarantee complete resolution. Common antibiotics used include clarithromycin, metronidazole, tetracycline, and amoxicillin.
  4. Nutritional support: Many individuals with Menetrier Disease suffer from malnutrition due to protein loss and impaired absorption. Nutritional interventions should aim at supplementing deficiencies and restoring overall health. This could involve enteral feeding through tubes, dietary modifications focused on high-calorie, easily digestible food intake, or intravenous infusions when needed.
  5. Surgery: Surgical intervention is usually reserved for severe cases where medical management has failed, persistent bleeding episodes occur, perforation risk exists, or significant weight loss is apparent. Subtotal gastrectomy involves removing part of the stomach, whereas near-total or total gastrectomy removes most or all of the organ. Surgery provides symptom relief but carries risks and potential side effects, making it a last resort option.
  6. Experimental treatments: Several experimental therapies show promise in managing Menetrier Disease, though they require further investigation before widespread adoption. Some novel agents target inflammatory markers, growth factor inhibition, or immune system modulation. For instance, monoclonal antibodies against TNF-alpha (infliximab) or IL-1 beta (canakinumab) may prove beneficial. Other investigational avenues include somatostatin analogs, tranexamic acid, and molecular targeted drugs.

What specialist should you speak with?

If you suspect having Menetrier Disease or would like professional advice about it, seek out specialists who possess extensive knowledge concerning gastrointestinal disorders and related conditions. Consulting a suitable expert ensures receiving accurate information and appropriate guidance tailored to specific concerns surrounding Menetrier Disease. Specialist recommendations include:

  1. Gastroenterologists: These physicians specialize in diagnosing and treating diseases impacting the digestive tract, including the stomach—the primary affected area in Menetrier Disease. Their proficiency covers evaluation techniques, therapeutic measures, and long-term management strategies, ensuring well-rounded assistance throughout diagnosis and post-diagnosis stages.
  2. Clinical geneticists: Although genetics play a minor role in Menetrier Disease development compared to environmental factors, consulting clinical geneticists may aid understanding any hereditary associations within families experiencing similar symptoms. Their expertise helps determine whether specific genes contribute significantly enough to warrant family screening initiatives.
  3. Internists: Internists hold broad training across adult medicine disciplines encompassing diverse body systems. Given Menetrier Disease’s multisystem implications beyond the gut itself (e.g., hypoalbuminemia, peripheral edema), internists serve as valuable allies providing holistic perspectives, integrative diagnostics, and coordinated care plans.
  4. Surgeons specialized in gastrointestinal procedures: Select cases necessitate surgical interventions, requiring collaboration between gastroenterologists and surgeons adept at operating around the abdomen. Such professionals evaluate operability thresholds based on severity levels, implement safe surgeries alleviating acute distress signals, and furnish postsurgical recovery protocols easing eventual lifestyle adjustments.

This article is written by

Dr. Emily Turner - gastroenterologist
Dr. Emily Turner - gastroenterologist
Dr. Emily Turner, MD, is a distinguished graduate of Johns Hopkins University School of Medicine, where she earned her medical degree. Her dedication to excellence led her to complete her residency at Massachusetts General Hospital, specializing in gastroenterology and digestive health.

Over the course of her illustrious career, Dr. Turner has held prominent roles at renowned medical institutions, including positions at Cleveland Clinic and Stanford University Medical Center. Her commitment to advancing patient care, combined with her research contributions, has solidified her reputation as a respected figure in the field.

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