A 35-year-old woman with celiac disease on a strict gluten-free diet for 2 years has persistent diarrhoea, weight loss, and jejunal biopsy showing persistent villous atrophy with increased intraepithelial lymphocytes (IELs). This most likely represents:
- A Non-compliance with gluten-free diet
- B Microscopic colitis concurrent with celiac disease
- C Refractory celiac disease type I or II ✓
- D Small intestinal bacterial overgrowth as a complication
Explanation
Refractory celiac disease (RCD) is defined by persistent symptoms and villous atrophy despite strict gluten-free diet for >12 months, after excluding non-compliance and other causes. RCD Type I has normal-phenotype IELs (CD3+CD8+); RCD Type II has aberrant IELs (CD3+CD8-surface, CD103-) which represent a clonal, pre-malignant population at high risk for enteropathy-associated T-cell lymphoma (EATL). Management requires specialist evaluation; RCD II requires surveillance and immunosuppression. Non-compliance is the most common cause, but 2 years of strict diet with persistent biopsy changes points to true RCD.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.