DDSEP® 8 Quick Quiz

February 2017 Quiz 2

Q2: A 34-year-old woman presents with a 3-year history of watery, nonbloody diarrhea with associated weight loss, and recurrent bacterial bronchitis and pneumonias. Laboratory studies show iron deficiency anemia, low 25-OH vitamin D, and a slightly elevated INR. Celiac serologies were negative, and small bowel biopsies revealed near total villous atrophy, increased intraepithelial lymphocytes, and crypt hyperplasia with absent plasma cells.

What is the most appropriate initial treatment strategy?

Gamma globulin

Prednisone

Infliximab

Gluten-free diet

Rifaximin

Q2: Answer: B

Objective: Recognize the features of CVID-associated noninfectious gastrointestinal manifestations.

Explanation: This patient has gastrointestinal manifestations of common variable immune deficiency (CVID), which can present similarly to celiac disease or inflammatory bowel disease. Histologically, intestinal biopsies will reveal villous atrophy, crypt hyperplasia, and intraepithelial lymphocytosis similar to celiac disease. However, while plasma cells are increased in celiac disease, they are absent in CVID.

The initial treatment strategy for CVID typically includes oral corticosteroids, either prednisone or budesonide, with other immunosuppressants such as the thiopurines or anti–tumor necrosis factor agents reserved for steroid-dependent or refractory disease.

Gluten-free diet is ineffective for the treatment of CVID-associated enteropathy. Intravenous immunoglobulin therapy reduces the frequency of infections associated with CVID, but does not affect the noninfectious GI symptoms. While bacterial overgrowth can occur in CVID, it is typically the consequence of the luminal changes, not the cause.

Reference

1. Agarwal S., Mayer L. Gastrointestinal manifestations in primary immune disorders. Inflamm Bowel Dis. 2010;16:703-11.

Next Article: