Nutritional remission without mucosal healing in type II refractory celiac disease: a case report

Type II refractory celiac disease (RCD II) is a rare pre-lymphomatous complication of celiac disease associated with severe malabsorption and a high risk of enteropathy-associated T-cell lymphoma. We report a 56-year-old Tunisian woman with adult-onset celiac disease who developed progressive diarrhea and life-threatening malnutrition despite dietitian-verified adherence to a gluten-free diet. At admission, she weighed 34 kg (body mass index, 12.9 kg/m²), was hypotensive and wheelchair dependent, and reported up to 20 stools/day. She had anemia, hypoalbuminemia, and hypophosphatemia. Computed tomography enterography, whole-body 18F-FDG PET/CT, jejunoscopy, and ileocolonoscopy found no lymphoma. Duodenal biopsies showed total villous atrophy and >50% aberrant CD3+/CD7+/CD8-/CD30- intraepithelial lymphocytes, supporting RCD II; flow cytometry and T-cell receptor clonality testing were unavailable. Treatment comprised 1 month of exclusive parenteral nutrition with refeeding-syndrome prophylaxis, 1 month of combined enteral and oral feeding, and subsequent oral nutrition. After systemic corticosteroids failed, open-capsule budesonide was initiated at 6 mg/day and reduced to 3 mg/day after 2 months. At 4 months, villous atrophy and the aberrant lymphocyte population were unchanged. At 6 months, however, the patient had gained 26 kg, stool frequency had fallen to 2-3/day, albumin had normalized, and mobility had improved. This case illustrates that nutritional recovery and intestinal disease activity may diverge in RCD II. Clinical and nutritional remission should not replace endoscopic and immunophenotypic surveillance.