Autoimmune enteropathy

From Libre Pathology
Jump to: navigation, search

Autoimmune enteropathy, abbreviated AIE, is a rare gastrointestinal pathology.

General

  • Considered a pediatric disease.
  • Super rare in adults - there are only ~11 reported cases in the literature.[1]
  • May be a component of the IPEX syndrome.[2]

Diagnosis is clinico-pathologic:[1]

  1. Intact immune system.
  2. Autoantibodies.
    • Anti-enterocyte antibody.
    • Anti-goblet antibody.
  3. Lack of response to gluten-free diet.

Microscopic

Features:[1]

  • +/-Loss of goblet cells.
  • +/-Loss of paneth cells.
  • Villous blunting.

DDx:

See also

References

  1. 1.0 1.1 1.2 Akram, S.; Murray, JA.; Pardi, DS.; Alexander, GL.; Schaffner, JA.; Russo, PA.; Abraham, SC. (Nov 2007). "Adult autoimmune enteropathy: Mayo Clinic Rochester experience.". Clin Gastroenterol Hepatol 5 (11): 1282-90; quiz 1245. doi:10.1016/j.cgh.2007.05.013. PMC 2128725. PMID 17683994. //www.ncbi.nlm.nih.gov/pmc/articles/PMC2128725/.
  2. Gentile, NM.; Murray, JA.; Pardi, DS. (Oct 2012). "Autoimmune enteropathy: a review and update of clinical management.". Curr Gastroenterol Rep 14 (5): 380-5. doi:10.1007/s11894-012-0276-2. PMID 22810979.