Autoimmune Enteropathy

Clinical

Usually presents <6 months of age
- Intractable diarrhea
- Non-responsive to TPN
- 30% mortality
Rarely presents in adults, usually with some other autoimmune disorder
- Many adult cases present as celiac disease that is not responsive to gluten free diet
- May be associated with thymoma
Extra-intestinal associations
- Insulin dependent diabetes mellitus
- Hypothyroidism
- Membranous glomerulonephritis
- Sclerosing cholangitis
- Autoimmune hepatitis
- Coombs+ hemolytic anemia
- Other autoantibodies
  - Anti-mitochondrial antibody
  - Anti-nuclear antibody
  - Anti-parietal cell antibody