Synopsis

 intractable diarrhea
 intestinal villous atrophy
 enteritis with intestinal chorionic chronic inflammation (inflammatory infiltrate of the lamina propria)
 +/- intestinal cryptic hyperplasia
 colitis (pancolitis)
 gastritis
 circulating anti-enterocyte antibodies (AEA)
 other auto-antibodies (anti-smooth muscle, anti-endoplasmic reticulum, anti-mitochondrial, anti-parietal cell, anti-adrenal, anti-DNA, anti-ANA)

Associations

 auto-immunity

• type I diabetes mellitus
• Coombs-positive auroimune hemolytic anemia
• neutropenia
• thrombocytopenia
• membranous glomerulonephritis
• interstitial nephritis
• dermatis herpetiformis
• hypothyroidy
• acute hepatitis
• chronic hepatitis
• diffuse interstitial pneumonitis
• bullous pemphigoid
• congenital alopecia
• systemic vasculitis (after immunosuppressive therapy) (#7726616#)
• chronic autoimmune hepatitis

 juvenile rheumatoid arthritis
 lymphoma and bone marrow transplantation

Variant

 familial autoimmune enteropathy (#7996356#)

See also

 IPEX syndrome (FOXP3 mutations)

References

 Ruemmele FM, Brousse N, Goulet O. Autoimmune enteropathy: molecular concepts. Curr Opin Gastroenterol. 2004 Nov;20(6):587-91. PMID: #15703687#

 Russo PA, Brochu P, Seidman EG, Roy CC. Autoimmune enteropathy. Pediatr Dev Pathol. 1999 Jan-Feb;2(1):65-71. PMID: #9841709#

 Jenkins HR, Jewkes F, Vujanic GM. Systemic vasculitis complicating infantile autoimmune enteropathy. Arch Dis Child. 1994 Dec;71(6):534-5. PMID: #7726616#