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Gastritis, Giant Hypertrophic


National Organization for Rare Disorders, Inc.

Synonyms

  • Gastroenteropathy, Protein Losing
  • Giant Hypertrophy of the Gastric Mucosa
  • Hypertrophic Gastropathy
  • Menetrier Disease

Disorder Subdivisions

  • None

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

  • Chronic Erosive Gastritis
  • Gastric Carcinoma
  • Eosinophilic Gastritis
  • Crohn's Disease
  • Non-Hodgkin Type Gastric Lymphoma
  • Cronkhite-Canada Syndrome
  • Amyloidosis
  • Ulcerative Colitis
  • Primary Sclerosing Cholangitis
  • Irritable Bowel Syndrome

General Discussion

Giant hypertrophic gastritis (GHG) is a general term for inflammation of the stomach due to the accumulation of inflammatory cells in the inner wall (mucosa) of the stomach resulting in abnormally large, coiled ridges or folds that resemble polyps in the inner wall of the stomach (hypertrophic gastric folds). GHG encompasses a collection of disorders. The symptoms of GHG may vary from case to case. The exact cause of GHG is unknown.

There is considerable confusion and contradiction in the medical literature regarding disorders involving large gastric folds. GHG is often used as a synonym for Menetrier disease. However, Menetrier disease is not a true form of gastritis. A diagnosis of Menetrier disease should indicate massive overgrowth of mucous cells (foveola) in the gastric mucosa (foveolar hyperplasia) and minimal inflammation. Foveolar hyperplasia results in large gastric folds. Because inflammation is minimal, Menetrier disease is classified as a form of hyperplastic gastropathy and not a form of gastritis. Some researchers believe that GHG and Menetrier disease may be variants of the same disorder or different parts of one disease spectrum.

Symptoms

The symptoms of giant hypertrophic gastritis may vary from case to case. Some individuals may not exhibit any symptoms (asymptomatic). Common symptoms that do occur include nausea, vomiting, diarrhea and pain in the upper middle region of the stomach (epigastric pain). In some cases, weight loss and profound loss of appetite (anorexia) may also occur.

Variable findings sometimes associated with GHG include low levels of the protein albumin (hypoalbuminemia), which may result in fluid accumulation (edema) in the stomach. Gastrointestinal bleeding has also been reported in some cases.

Causes

The exact cause of giant hypertrophic gastritis is unknown. Some researchers speculate that an exaggerated immune system response to an as yet unknown foreign or invading substance (antigen) may cause the disorder.

Individuals with GHG may also have another form of gastritis such as acute Helicobacter pylori gastritis. A form of GHG called lymphocytic gastritis occurs with greater frequency in children with a digestive disorder known as celiac sprue. However, the exact relationship, if any, among these disorders is not understood.

Some researchers have speculated that Helicobacter pylori infection plays a causative role in the development of GHG. However, no evidence has conclusively linked H. pylori infection to the development of GHG.

Affected Populations

Giant hypertrophic gastritis affects males and females in equal numbers. It can affect children or adults but occurs with greater frequency in late adulthood.

Related Disorders

Symptoms of the following disorders can be similar to those of giant hypertrophic gastritis. Comparisons may be useful for a differential diagnosis:

Menetrier disease is a rare disorder characterized by massive overgrowth of pits (foveola) in the gastric mucosa that are the openings for the gastric glands (foveolar hyperplasia) and minimal inflammation. Foveolar hyperplasia results in large gastric folds. The main symptom associated with Menetrier disease is pain in the upper middle region of the stomach (epigastric pain). Additional symptoms may include vomiting, nausea, weight loss, diarrhea, and gastrointestinal bleeding. Some individuals may have low levels of a protein called albumin (hypoalbuminemia), which may result in fluid accumulation (edema) in the stomach. The cause of Menetrier disease is unknown. (For more information on this disorder, choose "Menetrier" as your search term in the Rare Disease Database.)

Zollinger-Ellison syndrome (ZES) is a rare, digestive disorder characterized by the development of a tumor (gastrinoma) or tumors that secrete excessive levels of gastrin, a hormone that stimulates production of acid by the stomach. Many affected individuals develop multiple gastrinomas, approximately half to two-thirds of which may be cancerous (malignant). In most cases, the tumors arise within the pancreas and/or the upper region of the small intestine (duodenum). Due to excessive acid production (gastric acid hypersecretion), individuals with ZES may develop peptic ulcers of the stomach, the duodenum, and/or other regions of the digestive tract. Peptic ulcers are sores or raw areas within the digestive tract where the lining has been eroded by stomach acid and digestive juices. Symptoms and findings associated with ZES may include mild to severe abdominal pain; diarrhea; increased amounts of fat in the stools (steatorrhea); and/or other abnormalities. Individuals with Zollinger-Ellison syndrome may have giant gastric folds. (For more information on this disorder, choose "Zollinger-Ellison" as your search term in the Rare Disease Database.)

Additional causes of large gastric folds include Helicobacter pylori-associated gastritis, certain infections including syphilis and cytomegalovirus, and various forms of cancer include certain forms of carcinoma or lymphoma. Other rare disorders such as sarcoidosis or Cronkite-Canada disease may also be associated with large gastric folds. (For more information on these disorders, choose the specific disorder name as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
A diagnosis of giant hypertrophic gastritis may be suspected in individuals with large gastric folds. Large gastric folds may be diagnosed by an endoscopy, a procedure in which a thin, flexible tube (endoscope) is inserted through the mouth and used to examine the interior of the stomach and obtain tissue samples for microscopic study (biopsy).

A diagnosis of GHG may be confirmed based upon a thorough clinical evaluation, a detailed patient history, and the histiopathologic examination of affected stomach tissue, which shows characteristic accumulation of inflammatory cells in the gastric mucosa.

Treatment
There is no specific treatment for individuals with GHG. Treatment is symptomatic and supportive. In cases where H. pylori infection is documented affected individuals should receive antibacterial therapy.

Investigational Therapies

Recent studies suggest that the drug octreotide may be useful in reducing protein loss from the stomach associated with Giant Hypertrophic Gastritis. More study is needed to determine the long-term safety and effectiveness of this drug for the treatment of this disorder.

Some clinical researchers believe that Giant Hypertrophic Gastritis is the result of a stomach infection by the bacterium Helicobacter pylori, the agent that causes peptic ulcers. They are studying the effect of eliminating all traces of the bacterium from the stomachs of patients with the disorder.
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References

McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No:137280; Last Update:1/10/1994.

TEXTBOOKS
Merchant JL. Giant Hypertrophic Gastritis. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:341.

Fauci AS, et al., eds. Harrison's Principles of Internal Medicine, 14th Ed. New York, NY: McGraw-Hill, Inc; 1998:1610-3.

Beers MH, Berkow R, eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999.

Yamada T, et al., eds. Textbook of Gastroenterology. 2nd ed. Philadelphia, PA: J.B. Lippincott Company; 1995:1456-82.

JOURNAL ARTICLES
Weinstein WM. Emerging gastritides. Curr Gastroenterol Rep. 2001;3:523-7.

Niemela S, Karttunen TJ, Kerola T. Treatment of Helicobacter pylori in patients with lymphocytic gastritis. Hepatogastroenterology. 2001;48:1176-8.

Muller H, et al. Lymphocytic gastritis: a rare disorder of the gastric mucosa. Pathologe. 2001;22:56-61.

Hayat M, et al. Effects of Helicobacter pylori eradication on the natural history of lymphocytic gastritis. Gut. 1999;45:495-8.

Resources

NIH/National Digestive Diseases Information Clearinghouse
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Bethesda, MD 20892-3570
Tel: (301)654-3810
Fax: (301)907-8906
Tel: (800)891-5389
Email: nddic@info.niddk.nih.gov
Internet: http://www.niddk.nih.gov

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

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This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  9/10/2004
Copyright  1986, 1994, 2002, 2004 National Organization for Rare Disorders, Inc.



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