Mistakes in refractory coeliac disease and how to avoid them

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Mistakes in refractory coeliac disease and how to avoid them

Roberto De Giorgio, Giacomo Caio, Umberto Volta

Summary

AI Generated

Refractory coeliac disease is defined by persistent malabsorption and villous atrophy despite strict adherence to a gluten-free diet for over 12 months.

RCD diagnosis requires continuation or recurrence of symptoms and signs of malabsorption with villous atrophy in coeliac disease patients who have followed a strict gluten-free diet for more than 12 months
Serology in RCD is typically negative, though a small percentage of cases show positive results at low titre
Splenic hypofunction is a risk factor for RCD and can be detected by Howell–Jolly bodies and pitted red cells on peripheral blood smear or reduced spleen size on ultrasound
This material is relevant for gastroenterologists managing coeliac disease patients with persistent symptoms despite dietary compliance

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References

Mistakes

References

Mistake 1 Mistake 2 Mistake 3 Mistake 4 Mistake 5 Mistake 6 Mistake 7 Mistake 8 Mistake 9 Mistake 10

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van Wanrooij RLJ, Bouma G, Bontkes HJ, et al. Outcome of referrals for non-responsive celiac disease in a tertiary center: Low incidence of refractory celiac disease in the Netherlands. Clin Transl Gastroenterol 2017; 8: e218. [Link]

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10.

Jamma S, Leffler DA, Dennis M, et al. Small intestinal release mesalamine for the treatment of refractory celiac disease type I. J Clin Gastroenterol 2011; 45: 30–33. [Link]

11.

Al-Toma A, Visser OJ, van Roessel HM, et al. Autologous hematopoietic stem cell transplantation in refractory celiac disease with aberrant T cells. Blood 2007; 109: 2243–2249. [Link]

12.

Cellier C, Bouma G, van Gils T et al. [AGA Abstract 616] AMG 714 (ANTI-IL-15 MAB) halts the progression of aberrant intraepithelial lymphocytes in refractory celiac disease type ii (RCD-II): A phase 2a, randomized, double-blind, placebo-controlled study evaluating AMG 714 in adult patients with RCD-II/PRE-EATL. Gastroenterology 2018; 154 (6) Suppl 1: S-129–S-130. [Link]

13.

Nijeboer P, van Wanrooij R, van Gils T, et al. Lymphoma development and survival in refractory coeliac disease type II: Histological response as prognostic factor. United Eur Gastroenterol J 2017; 5: 208–217. [Link]

14.

Verbeek WHM, Goerres MS, von Blomberg BME, et al. Flow cytometric determination of aberrant intra-epithelial lymphocytes predicts T-cell lymphoma development more accurately than T-cell clonality analysis in refractory celiac disease. Clin Immunol 2008; 126: 48–56. [Link]

15.

Al-Toma A, Volta U, Auricchio R, et al. European Society for the Study of Coeliac Disease (ESsCD) guideline for coeliac disease and other gluten-related disorders. United Eur Gastroenterol J 2019; 7: 583–613. [Link]

16.

Roma E, Roubani A, Kolia E, et al. Dietary compliance and life style of children with coeliac disease. J Hum Nutr Diet. 2010; 23: 176–182. [Link]

17.

Aziz I, Peerally MF, Barnes JH, et al. The clinical and phenotypical assessment of seronegative villous atrophy: a prospective UK centre experience evaluating 200 adult cases over a 15-year period (2000–2015). Gut 2017; 66: 1563–1572. [Link]

18.

Wahab PJ, Meijer JW and Mulder CJ. Histologic follow-up of people with celiac disease on a gluten-free diet: slow and incomplete recovery. Am J Clin Pathol 2002; 118: 459–463. [Link]

19.

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20.

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Abstract

Refractory coeliac disease (RCD) is characterized by the persistence or recurrence of symptoms and signs of malabsorption associated with villous atrophy in patients with coeliac disease who have adhered to a strict gluten-free diet (GFD) for more than 12 months.^1–3 Serology is usually negative or, in a small percentage of cases, positive at a low titre.⁴ Splenic hypofunction, a risk factor for RCD, can be indicated by Howell–Jolly bodies and pitted red cells in a peripheral blood smear. A reduced spleen size visible on ultrasound examination also provides direct evidence of hyposplenism.⁵

Topics

Small Intestine & Nutrition

Citation

Volta U, Caio G and De Giorgio R. Mistakes in refractory coeliac disease and how to avoid them. UEG Education 2019; 19: 15–18.

Published

2025

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