DDSEP® 8 Quick Quiz

Question 1

Publish date: March 31, 2021

Q1. A 55-year-old woman presents with a one-year history of large volume foul-smelling stools that float in water associated with 40-pound weight loss. Laboratory evaluation reveals low vitamin A and D levels. An upper endoscopy with duodenal biopsies reveals complete villous blunting with decreased goblet and Paneth cells, absence of surface intraepithelial lymphocytes, and increased crypt apoptosis. She denies nonsteroidal anti-inflammatory drug use, celiac serologies were not elevated, and a glucose hydrogen breath test was negative. She also has coexisting rheumatoid arthritis and multiple sclerosis.

Which of the following tests can help establish the diagnosis?

Antinuclear antibodies

Anti-smooth muscle antibodies

Anti-gastric parietal cell antibodies

Antienterocyte antibodies

Antithyroglobulin antibodies

Correct answer: D. Antienterocyte antibodies


Rationale
Autoimmune enteropathy (AIE) is characterized by a severe malabsorption and secretory diarrhea, and is differentiated from celiac disease on small-bowel biopsy by the decreased numbers or absence of surface intraepithelial lymphocytes, apoptotic bodies present in the intestinal crypts, and absent goblet and Paneth cells. Patients with AIE may also carry other autoimmune conditions such as rheumatoid arthritis and multiple sclerosis. A group at the Mayo Clinic has published a set of diagnostic criteria based on their case series of adult AIE that requires ruling out other causes of chronic diarrhea in adults, specific histology supportive of AIE, and presence of malabsorption and ruling out other causes of villous atrophy. The presence of antienterocyte or antigoblet cell antibodies are supportive of a diagnosis of AIE, but their absence does not exclude the diagnosis.

References
Akram S et al. Clin Gastroenterol Hepatol. 2007;5(11):1282-90.
Montalto M et al. Scan J Gastroenterol. 2009;44(9):1029-36.

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