Celiac Disease: Ten Things That Every Gastroenterologist Should Know

Amy S. Oxentenko; Joseph A. Murray


Clin Gastroenterol Hepatol. 2015;13(8):1396-1404. 

In This Article

10. What Do We Do With Refractory Celiac Disease?

RCD is defined as recurrent or persistent villous atrophy associated with malabsorption despite 12 months or more on a verified GFD in patients with CD, without other diagnoses or gluten contamination.[76] RCD affects only 1.5% of CD patients.[77] RCD is divided into 2 types. RCD type I is defined as those who meet the definition of RCD, with polyclonal intraepithelial lymphocytes bearing typical CD3 and CD8 surface markers, whereas RCD type II has clonal T-cell receptors, without the typical CD3–T-cell receptor surface markers, but preserved intracellular CD3 expression.[76] Of those with RCD, approximately 15% are RCD type II.[77] Prognosis in RCD II is poor, with 5-year survival at 44%–58%[78–80] and a high risk of lymphoma.[80] To evaluate for RCD, duodenal biopsies should undergo immunophenotyping and T-cell rearrangement studies. RCD, ulcerative jejunitis, and EATL are complications of CD that need to be considered carefully in NRCD.

Practical Suggestion

Ongoing villous atrophy should prompt immunophenotyping and T-cell rearrangement studies to look for RCD II and lymphoma. RCD prompts further highly specialized investigations, including determination of type and ongoing close surveillance for EATL.