New Treatment Option for Autoimmune Enteropathy: A Rare Case of Intractable Diarrhea Treated with Vedolizumab

Abstract

Autoimmune enteropathy is an uncommon cause of chronic diarrhea rarely seen in adults. The disease is secondary to an autoimmune process in the gut that leads to villous blunting and subsequent watery diarrhea, abdominal pain, and severe weight loss. The disease has only been described in 37 adults prior to our case, and variable treatment success has been documented with steroids, immunomodulators, and TNF-α inhibitors. This case is the first to show success in treating autoimmune enteropathy with vedolizumab and provides physicians with an additional therapeutic option when limited by a patient's comorbidities and side effects of other drugs.

Figure 1

Endoscopic picture from colonoscopy in 2016 revealing diffusely edematous mucosa.

Figure 2

The patient’s 2015 biopsy revealed (A) villous blunting, increased lamina propria lymphoplasmacytic infiltrate, increased epithelial lymphocytes in the duodenum, and absence of goblet cells; and (B) increased lamina propria lymphoplasmacytic infiltrate and an absence of goblet cells in the colon. Patchy neutrophilic cryptitis and a marked increase in intraepithelial crypt apoptosis are also seen.

Figure 3

Endoscopic picture taken from the patient's most recent colonoscopy depicting a less inflamed but still edematous mucosa.

Figure 4

(A) Recent biopsy of the duodenum showing findings similar to those of previous biopsies, though with some improvement in the degree of inflammation and the amount of lymphoplasmacytic lamina propria inflammation. Note the more intact villous architecture of the duodenum. (B) Recent biopsy of the colon showing findings similar to prior biopsies with improvement in the amount of lymphoplasmacytic lamina propria inflammation and neutrophilic cryptitis. Increased crypt epithelial apoptoses are still present. A paucity of goblet cells remains, but they are increased in number compared to previous biopsies.