Celiac Disease: 10 Things a Gastroenterologist Should Know
Celiac Disease: 10 Things a Gastroenterologist Should Know
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. RCD affects only 1.5% of CD patients. 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. Of those with RCD, approximately 15% are RCD type II. Prognosis in RCD II is poor, with 5-year survival at 44%–58% and a high risk of lymphoma. 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.
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.
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. RCD affects only 1.5% of CD patients. 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. Of those with RCD, approximately 15% are RCD type II. Prognosis in RCD II is poor, with 5-year survival at 44%–58% and a high risk of lymphoma. 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.
Source...