GASTROENTEROLOGY ARTICLE OF THE WEEK

October 12, 2000

 Ryan BM, Kelleher D.  Refractory Celiac Disease.  Gastroenterology 2000;119:243-251.

 1.  Which of the following cases may be classified as refractory sprue?

            a.  a patient who improves symptomatically on GFD, but repeat small bowel

biopsies 3 months later still show histologic changes of sprue

            b.  a patient with no clinical or histologic improvement after 14 months of GFD

            c.  a teenager who improves on GFD, but symptoms persist, EMA titers continue

to be positive, histology improved but not normalized after 12 months.

            d.  a woman who becomes asymptomatic on GFD, histology reverts to normal,

and she has a symptomatic and histologic relapse despite strict adherence to diet.

 2.  Characteristic histologic lesions of celiac disease include

            a.  lipid droplets in villi

            b.  intraepithelial lymphocytes

            c.  crypt hyperplasia

            d.  foamy macrophages

            e.  villous blunting

            f.   a pathognomonic histologic picture

 True or False

 3.  Intestinal lymphoma may mimic refractory sprue, laparotomy with full thickness biopsy of the bowel may be needed to exclude this diagnosis.

 4.  Refractory sprue may be a manifestation of an underlying autoimmune enteropathy

 5.  Patients with refractory sprue may indeed have small bowel B-cell lymphoma 

6.  The prevalence of refractory sprue among patients with celiac disease is estimated to be about 7% to 8%

 7.  Among patients with celiac disease, histologic improvement in response to gluten free diet is usually evident and complete by 4 to 6 weeks after initiation of diet.

 8.  HLA-DQ2 allele is found in almost all patients with celiac disease.

 9.  Treatment options for refractory sprue may include

            a.  corticosteroids, which may mask signs and symptoms of lymphoma

            b.  exclusion diets

            c.  replacement of trace elements, parenteral nutrition if required

            d.  azathioprine which is usually effective in steroid-refractory cases

            e.  cyclosporine which may increase risk for opportunistic infections, or facilitate

an overt presentation of a cryptic T-cell lymphoma.

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