Rare Medical News

Disease Profile

Autoimmune gastrointestinal dysmotility

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.

Unknown

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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Other names (AKA)

AGID

Categories

Autoimmune / Autoinflammatory diseases; Digestive Diseases

Summary

Autoimmune gastrointestinal dysmotility (AGID) is a type of dysautonomia that may be idiopathic (cause unknown) or associated with cancer elsewhere in the body, most commonly small cell lung cancer.[1] Signs and symptoms may include early satiety (feeling full quickly), nausea, vomiting, bloating, diarrhea, constipation and involuntary weight loss.[2] Management options for AGID include treating specific symptoms, treatment of any underlying cancer if necessary, and/or immunotherapy. Nutrition and hydration therapy are important supportive treatment measures.[3]

Treatment

Treatment options for AGID are limited and generally aim to the relieve specific symptoms in each person.[2] Therapies that may be useful include anti-emetics (drugs that relieve nausea and vomiting), prokinetic agents (e.g., erythromycin) to enhance motility, and cholinesterase inhibitors. Improvement of symptoms has also been reported with immunotherapy (intravenous immune globulin) and plasma exchange.[2][3] Other types of treatments may be useful depending on symptoms and severity. Maintenance of nutrition and hydration, treatment of small-bowel bacterial overgrowth, and management of abdominal pain are important supportive measures.[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.

    In-Depth Information

    • PubMed is a searchable database of medical literature and lists journal articles that discuss Autoimmune gastrointestinal dysmotility. Click on the link to view a sample search on this topic.

      References

      1. Camilleri M. Gastroparesis: Etiology, clinical manifestations, and diagnosi. UpToDate. Waltham, MA: UpToDate; June, 2017; https://www.uptodate.com/contents/gastroparesis-etiology-clinical-manifestations-and-diagnosis.
      2. Flanagan EP, Saito YA, Lennon VA, et. al. Immunotherapy trial as diagnostic test in evaluating patients with presumed autoimmune gastrointestinal dysmotility. Neurogastroenterol Motil. September, 2014; 26(9):1285-1297. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4149849/.
      3. Pasha SF, Lunsford TN, Lennon VA. Autoimmune gastrointestinal dysmotility treated successfully with pyridostigmine. Gastroenterology. 2006; 131(5):1592-6. https://www.ncbi.nlm.nih.gov/pubmed/17101331.

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