Rare Pulmonology News

Disease Profile

Autoimmune atrophic gastritis

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

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Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Summary

Autoimmune atrophic gastritis is a chronic inflammatory disease in which the immune system mistakenly destroys a special type of cell (parietal cells) in the stomach. Parietal cells make stomach acid (gastric acid) and a substance our body needs to help absorb vitamin B12 (called intrinsic factor). The progressive loss of parietal cells may lead to iron deficiency and finally vitamin B12 deficiency. The clinical signs and symptoms of iron deficiency anemia include tiredness, pale complexion, and heart problems such as exercise intolerance and palpitations. B12 deficiency may lead to pernicious anemia as well as gastrointestinal and neurological problems. Autoimmune atrophic gastritis may also be associated with an increased risk of certain types of stomach cancers.[1][2][3]

The cause of autoimmune gastritis is unknown, but affected people are likely to have other autoimmune disorders including autoimmune thyroiditis, diabetes type I, Addison’s disease, and vitiligo. Diagnosis is made through a combination of clinical findings (certain blood tests and presence of other autoimmune conditions) and biopsy of stomach lining. Treatment is based on the signs and symptoms present in each person, but may include iron infusions, vitamin B12 injections and endoscopic surveillance.[1][2][3]

Symptoms

In some cases, autoimmune atrophic gastritis does not cause any obvious signs and symptoms. However, some people may experience nausea, vomiting, a feeling of fullness in the upper abdomen after eating, or abdominal pain. It is often associated with impaired absorption of vitamin B12 and possibly other vitamin deficiencies (such as folate and iron). People with vitamin B12 deficiency are at risk for pernicious anemia, a condition in which the body does not have enough healthy red blood cells.[4][5]

Autoimmune atrophic gastritis is considered a "precancerous" condition and it may be responsible for the development of gastric adenocarcinoma or carcinoids.[6]

Cause

Autoimmune atrophic gastritis is considered an autoimmune disorder. In people who are affected by this condition, the immune system mistakenly attacks the healthy cells of the stomach lining. Overtime, this can wear away the stomach's protective barrier and interfere with the absorption of several key vitamins (i.e. vitamin B12, iron, folate). This leads to the signs and symptoms of autoimmune atrophic gastritis.[4][7]

Diagnosis

A diagnosis of autoimmune atrophic gastritis is generally not suspected until characteristic signs and symptoms are present. Additional testing can then be ordered to confirm the diagnosis. This generally includes:[1][8]

  • A biopsy of the affected tissue obtained through endoscopy
  • Blood work that demonstrates autoantibodies against certain cells of the stomach

Treatment

The treatment of autoimmune atrophic gastritis is generally focused on preventing or treating vitamin B12 and iron deficiencies. If pernicious anemia is already present at the time of diagnosis, vitamin B12 shots (injections) may be recommended. Since dietary and oral iron supplements do not usually improve iron levels, alternative iron therapy approaches may include receiving periodic intravenous (IV) iron (iron infusion) to increase iron stores or a daily dose of oral ferrous glycine sulfate to meet daily iron requirements. People with autoimmune atrophic gastritis should have their levels of B12 and iron monitored for the rest of their life.[1][2][3]

In some cases, periodic endoscopy may also be recommended due to the increased risk of certain types of cancer.[1][2][3]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Providing General Support

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • The Merck Manual for health care professionals provides information on Autoimmune atrophic gastritis.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Autoimmune atrophic gastritis. Click on the link to view a sample search on this topic.

        References

        1. Park JY, Lam-Himlin D, Vemulapalli R. Review of autoimmune metaplastic atrophic gastritis. Gastrointest Endosc. February 2013; 77(2):284-292. https://www.ncbi.nlm.nih.gov/pubmed/23199649.
        2. Miguel N, Costa E, Santalha M Jr, Lima R, Vizcaino JR, Pereira F, and Barbot J. Refractory Iron-deficiency Anemia and Autoimmune Atrophic Gastritis in Pediatric Age Group: Analysis of 8 Clinical Cases. J Pediatr Hematol Oncol. March 2014; 36(2):134-9. https://www.ncbi.nlm.nih.gov/pubmed/24327126.
        3. Kulnigg-Dabsch S. Autoimmune gastritis. Wiener Medizinische Wochenschrift. September 26 2016; 166(13):424-430. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5065578/.
        4. Nafea Zayouna, MD. Atrophic Gastritis. Medscape Reference. December 2014; https://emedicine.medscape.com/article/176036-overview.
        5. Pamela J Jensen, MD; Mark Feldman, MD, MACP, AGAF, FACG. Metaplastic (chronic) atrophic gastritis. UpToDate. December 17 2015; https://www.uptodate.com/contents/metaplastic-chronic-atrophic-gastritis.
        6. Miceli E, Lenti MV, Padula D, Luinetti O, Vattiato C, Monti CM, Di Stefano M, Corazza GR. Common features of patients with autoimmune atrophic gastritis. Clin Gastroenterol Hepatol. July 2012; 10(7):812-814.
        7. Gastritis. National Institute of Diabetes and Digestive and Kidney Diseases. July 2015; https://www.niddk.nih.gov/health-information/health-topics/digestive-diseases/gastritis/Pages/facts.aspx#signs.
        8. Neumann WL, Coss E, Rugge M, Genta RM. Autoimmune atrophic gastritis--pathogenesis, pathology and management. Nat Rev Gastroenterol Hepatol. September 2013; 10(9):529-541. https://www.ncbi.nlm.nih.gov/pubmed/23774773.

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