Autoimmune enteropathy

Autoimmune enteropathy
Other namesSevere immune-mediated enteropathy, Immune-mediated protracted diarrhea of infancy
Histological evidence of enteropathy (inflammatory infiltrate, villus blunting) seen in this intestinal biopsy from a child with malnutrition.
SpecialtyImmunology Gastroenterology
SymptomsDiarrhea, and autoimmune damage to the intestinal mucosa.[1]
ComplicationsElectrolyte imbalances, malabsorption, and failure to thrive.[2]
Usual onsetFirst six months of life.[3]
DurationLifelong[4]
Diagnostic methodhistological changes, serologic testing, and clinical signs and symptoms.[5]
Differential diagnosisGraft-versus-host disease, Crohn's disease, celiac disease and lactose intolerance.[6]
TreatmentParenteral nutrition and corticosteroids.[7]
Prognosis30% mortality rate without treatment.[8]
Frequency<1 in 100,000 infants.[1]

Autoimmune enteropathy is a rare autoimmune disorder characterized by weight loss from malabsorption, severe and protracted diarrhea, and autoimmune damage to the intestinal mucosa.[1] Autoimmune enteropathy typically occurs in infants and younger children however, adult cases have been reported in literature.[9] Autoimmune enteropathy was first described by Walker-Smith et al. in 1982.[10]

The mechanisms of autoimmune enteropathy isn't well known but dysfunction or deficiency of CD25+CD4+ regulatory T cells may play a role.[11] Numerous other illnesses and syndromes are linked to autoimmune enteropathy, the most prominent being Autoimmune polyendocrine syndrome type 1 and immune dysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome.[5]

Clinical symptoms, laboratory results, and the histological characteristics of a small bowel biopsy are used to make the diagnosis.[5] These patients typically don't respond to diet modification and often require immune-suppressants and sometimes require total parenteral nutrition.[12] The prevalence of autoimmune enteropathy is estimated to be less than 1 in 100,000 infants[1]

The prognosis of autoimmune enteropathy varies and depends on systemic manifestations, the severity of symptoms, and the degree of gastrointestinal involvement.[13] Children suffering from autoimmune enteropathy are frequently vulnerable to systemic and local infections pertaining to immunotherapy, the intestinal and skin barriers, and malnourishment.[14]

  1. ^ a b c d Montalto, Massimo; D'Onofrio, Ferruccio; Santoro, Luca; Gallo, Antonella; Gasbarrini, Antonio; Gasbarrini, Giovanni (2009). "Autoimmune enteropathy in children and adults". Scandinavian Journal of Gastroenterology. 44 (9). Informa UK Limited: 1029–1036. doi:10.1080/00365520902783691. ISSN 0036-5521. PMID 19255930. S2CID 33004674. Retrieved December 1, 2023.
  2. ^ Cite error: The named reference Ruemmele Brousse Goulet 2004 pp. 587–591 was invoked but never defined (see the help page).
  3. ^ Cite error: The named reference Focus on Stem Cell Transplant Therapy was invoked but never defined (see the help page).
  4. ^ Cite error: The named reference Autoimmune enteropathies was invoked but never defined (see the help page).
  5. ^ a b c Chen, Charles B.; Tahboub, Farah; Plesec, Thomas; Kay, Marsha; Radhakrishnan, Kadakkal (August 24, 2020). "A Review of Autoimmune Enteropathy and Its Associated Syndromes". Digestive Diseases and Sciences. 65 (11). Springer Science and Business Media LLC: 3079–3090. doi:10.1007/s10620-020-06540-8. ISSN 0163-2116. PMID 32833153. S2CID 221257881. Retrieved December 1, 2023.
  6. ^ Cite error: The named reference Pediatric autoimmune enteropathy was invoked but never defined (see the help page).
  7. ^ Cite error: The named reference From autoimmune enteropathy to the IPEX was invoked but never defined (see the help page).
  8. ^ Cite error: The named reference Successful treatment with cyclosporine was invoked but never defined (see the help page).
  9. ^ Biagi, F; Corazza, GR (1997). "Autoimmune enteropathy in adults". The Lancet. 350 (9082). Elsevier BV: 960. doi:10.1016/s0140-6736(05)63302-8. ISSN 0140-6736.
  10. ^ WALKERSMITH, J (1982). "Autoantibodies Against Gut Epithelium in Child with Small-Intestinal Enteropathy". The Lancet. 319 (8271). Elsevier BV: 566–567. doi:10.1016/s0140-6736(82)92076-1. ISSN 0140-6736. PMID 6120421. S2CID 26473878.
  11. ^ Sakaguchi, Shimon (March 22, 2005). "Naturally arising Foxp3-expressing CD25+CD4+ regulatory T cells in immunological tolerance to self and non-self". Nature Immunology. 6 (4). Springer Science and Business Media LLC: 345–352. doi:10.1038/ni1178. ISSN 1529-2908. PMID 15785760. S2CID 14683763.
  12. ^ Akram, Salma; Murray, Joseph A.; Pardi, Darrell S.; Alexander, Glenn L.; Schaffner, John A.; Russo, Pierre A.; Abraham, Susan C. (2007). "Adult Autoimmune Enteropathy: Mayo Clinic Rochester Experience". Clinical Gastroenterology and Hepatology. 5 (11). Elsevier BV: 1282–1290. doi:10.1016/j.cgh.2007.05.013. ISSN 1542-3565. PMC 2128725. PMID 17683994.
  13. ^ Hill, S M; Milla, P J; Bottazzo, G F; Mirakian, R (January 1, 1991). "Autoimmune enteropathy and colitis: is there a generalised autoimmune gut disorder?". Gut. BMJ. 32 (1): 36–42. doi:10.1136/gut.32.1.36. ISSN 0017-5749. PMC 1379210. PMID 1991636.
  14. ^ Cuenod, Bana; Brousse, Nicole; Goulet, Olivier; De Potter, Sophie; Mougenot, Jean-François; Ricour, Claude; Guy-Grand, Delphine; Cerf-Bensussan, Nadine (1990). "Classification of intractable diarrhea in infancy using clinical and immunohistological criteria". Gastroenterology. 99 (4). Elsevier BV: 1037–1043. doi:10.1016/0016-5085(90)90624-a. ISSN 0016-5085. PMID 2203660.
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