Refractory celiac disease type 2 (RCD2) is a rare and severe complication in which malabsorptive symptoms and small-intestinal villous atrophy persist despite strict adherence to a gluten-free diet for more than 12 months. The presence of an abnormal clonal intestinal T-cell population distinguishes RCD type 2 from type 1 and marks a clinically distinct and high-risk subgroup.
The patient has confirmed RCD type 2: persistent or recurrent malabsorption with small-intestinal villous atrophy after more than 12 months of strict gluten-free diet, together with a clonal or abnormal intestinal T-cell population — evidenced by increased CD3-positive intraepithelial lymphocytes lacking CD8 expression or a clonal T-cell receptor gene rearrangement. RCD type 2 carries a poorer prognosis than type 1 and is recognised as a precursor to enteropathy-associated T-cell lymphoma.
Management of RCD type 2 moves beyond dietary modification and involves specific pharmacological agents targeted at the abnormal T-cell population, as well as — in carefully selected patients — advanced interventional strategies. Nutritional support is a core component of the overall management plan. The complete regimen, including all options and their sequencing, is in the full protocol.
DOI: 10.14309/ajg.0000000000002075