|55 Kenosia Avenue
Danbury, CT 06810
Toll Free: 1.800.999.6673
The National Organization for Rare Disorders (NORD) web site, its databases, and the contents thereof are copyrighted by NORD. No part of the NORD web site, databases, or the contents may be copied in any way, including but not limited to the following: electronically downloading, storing in a retrieval system, or redistributing for any commercial purposes without the express written permission of NORD. Permission is hereby granted to print one hard copy of the information on an individual disease for your personal use, provided that such content is in no way modified, and the credit for the source (NORD) and NORD’s copyright notice are included on the printed copy. Any other electronic reproduction or other printed versions is strictly prohibited.
The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.
Copyright 1986, 1994, 2002, 2004
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.
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.
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.
Giant hypertrophic gastritis affects males and females in equal numbers. It can affect children or adults but occurs with greater frequency in late adulthood.
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.)
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.
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.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
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.
Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.
Merchant JL. Giant Hypertrophic Gastritis. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:341.
Beers MH, Berkow R, eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999.
Fauci AS, et al., eds. Harrison's Principles of Internal Medicine, 14th Ed. New York, NY: McGraw-Hill, Inc; 1998:1610-3.
Yamada T, et al., eds. Textbook of Gastroenterology. 2nd ed. Philadelphia, PA: J.B. Lippincott Company; 1995:1456-82.
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.
McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No:137280; Last Update:1/10/1994.
Report last updated: 2008/04/24 00:00:00 GMT+0