A 45-year-old man with known coeliac disease on a strict gluten-free diet for 3 years continues to have diarrhoea and weight loss. Repeat duodenal biopsy shows villous atrophy persisting. Anti-tTG IgA remains elevated. He admits to accidental gluten exposure. However, a third biopsy after further dietary restriction shows intraepithelial lymphocytosis, villous atrophy, and aberrant CD3+ CD8- intraepithelial lymphocytes on flow cytometry. What complication should be considered?

• A Simple dietary non-compliance; repeat dietary counselling
• B Collagenous sprue; treat with budesonide
• C Small intestinal bacterial overgrowth complicating coeliac disease
• D Refractory coeliac disease type II (RCD-II) with risk of enteropathy-associated T-cell lymphoma (EATL) ✓

Explanation

Refractory coeliac disease type II is defined by persistence of villous atrophy despite strict gluten-free diet with a clonal population of aberrant intraepithelial lymphocytes (CD3+CD8−CD103+) that lack surface TCR. This represents a pre-malignant condition with significant risk of transformation to enteropathy-associated T-cell lymphoma (EATL), the most feared complication of coeliac disease. Investigation with CT/PET and referral to specialist centre is mandatory.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.