Diagnosis and management of refractory celiac disease: a systematic review

##plugins.themes.academic_pro.article.main##

Asma Labidi
Meriem Serghini
Sami Karoui
Jalel Boubaker
Azza Filali

Abstract

Background: Refractory celiac disease is defined by persisting malabsorptive symptoms in spite of a strict gluten free diet for at least 6 to 12 months. Alternatives to gluten free diet seem to be still controversial.
aim: To describe the clinical and epidemiologic aspects of refractory celiac disease, and to identify therapeutic options in this condition.
methods: Systematic review and critical analysis of observational studies, clinical trials and case reports that focused on diagnosis and management of refractory celiac disease.
results: Refractory celiac disease can be classified as type 1 or type 2 according to the phenotype of intraepithelial lymphocytes. Great complications such as enteropathy-associated T-cell lymphoma may occur in a subgroup of these patients mainly in refractory celiac disease type 2. Curative therapies are still lacking.
Conclusion: Refractory celiac disease remains a diagnosis of exclusion. Its prognosis remains still dismal by the absence yet of curative therapies. However, some new treatments seem to hold promise during few cohort-studies.

Keywords:

Celiac disease; refractory celiac disease

##plugins.themes.academic_pro.article.details##

References

  1. Gee S. On the coeliac affection. St Bartholomew's Hospital Reports 1888;24:17-20.
  2. National Institutes of Health Consensus Development Conference Statement on Celiac Disease, Gastroenterology 2005;128:1-9
  3. Ben Hariz M , Kallel-Sellami M, Kallel L, et al. Prevalence of celiac disease in Tunisia: mass-screening study in schoolchildren. Eur J Gastroenterol Hepatol. 2007;19:687-94
  4. Daum S, Cellier C, Mulder CJ. Refractory coeliac disease. Best Pract Res Clin Gastroenterol 2005;19:413-24.
  5. Ryan BM, Kelleher D. Refractory celiac disease. Gastroenterology 2000;119:243-51.
  6. Potter DD, Murray JA, Donohue JH, et al. The role of defective mismatch repair in small bowel adenocarcinoma in celiac disease. Cancer Res 2004;64:7073-7.
  7. Cellier C, Delabesse E, Helmer C, et al. Refractory sprue, coeliac disease, and enteropathy-associated T-cell lymphoma. Lancet 2000;356:203-8.
  8. Abdulkarim AS, Burgart LJ, See J, Murray JA. Etiology of nonresponsive celiac disease: results of a systematic approach. Am J Gastroenterol 2002;97:2016-21.
  9. Alberto Rubio-Tapia, Joseph A Murray. Classification and Management of Refractory Celiac Disease, Gut 2010 ; 59:547- 57.
  10. Malamut G, Afchain P, Verkarre V et al. Presentation and longterm follow-up of refractory celiac disease: comparison of type I with type II. Gastroenterology 2009;136 :81-90.
  11. Lewis NR, Scott BB. Systematic review: the use of serology to exclude or diagnose coeliac disease: a comparison of the endomysial and tissue transglutaminase antibody tests. Aliment Pharmacol Ther 2006;24:47-54
  12. Wahab P, Meijer J.W.R, Mulder C.J.J. Histologic follow up of people with celiac disease on a gluten-free diet: slow and incomplete recovery. Am J Clin Pathol 2002; 118: 459-63.
  13. Kitis G, Holmes GK, Cooper BT et al. Association of celiac disease and inflammatory bowel disease. Gut 1980; 21: 636-41.
  14. Kalha I, Sellin JH. Common variable immunodeficiency and the gastrointestinal tract. Curr Gastroenterol Rep 2004; 6: 377-83.
  15. Daum S, Ulirich R, Heise W, et al. Intestinal non-Hodgkin's lymphoma: a multicenter prospective clinical study from the German Study Group on Intestinal non-Hodgkin's Lymphoma. J Clin Oncol 2003; 21: 2740-6.
  16. Olaussen R, Lovik A, Andersen P, et al. Elemental diet reduces the frequency of interferon-gammasecreting mucosal T-cells in refractory celiac disease. Best Practice and research Clinical Gastrology 2005;19: 413-24.
  17. See J, Murray J A. Gluten-free diet: the medical and nutrition management of celiac disease. Nutr Clin Pract 2006;21:1-15.
  18. Haines ML, Anderson RP, Gibson PR. Systematic review: The evidence base for long-term management of coeliac disease. Aliment Pharmacol Ther 2008;28:1042-66
  19. Cellier C, Delabesse E, Helmer C et al. Refractory sprue, celiac disease, and enteropathy-associated T-cell lymphoma. Lancet 2000; 356: 203-8.
  20. Alfred J, Adrian P, Booth W, Pearse A. Response of the jejunal mucosa in adult celiac disease to oral prednisolone. .Gut 1970;11:7-14.
  21. Mitchison H C, Mardini H Al, Gillespie S, Laker M, Zaitoun A, Record C. A pilot study of fluticasone propionate in untreated coeliac disease. Gut 1991;32:260-5.
  22. Ryan B.M, Kelleher D. Refractory celiac disease. Gastroenterology 2000;119:243-51
  23. Peters TJ, Jones PE, Jenkins WJ, Wells G. Analytical subcellular fractionation of jejunal biopsy specimens: enzyme activities, organelle pathology and response to corticosteroids in patients with non-responsive celiac disease. Clin Sci Mol Med 1978;55:293-300.
  24. Cellier C, Brousse N, Cerf-Bensussan N. Classification and outcome of refractory sprue. Lancet 2000;356 :203-8.
  25. Brar P, Lee S, Lewis S, Egbuna I, Bhagat G, Green PH. Budesonide in the treatment of refractory celiac disease. Am J Gastroenterol. 2007;102:2265-9.
  26. Goerres MS, Meijer JW, Wahab PJ et al. Azathioprine and prednisone combination therapy in refractory celiac disease. Aliment Pharmacol Ther 2003;18:487-94.
  27. Wahab PJ, Crusius JB, Meijer JW, Uil JJ, Mulder CJ. Cyclosporin in the treatment of adults with refractory celiac disease-an open pilot study. Aliment Pharmacol Ther 2000; 14: 767-74.
  28. Gillett H.R, Arnott DR, McIntyre M et al. Successful infliximab treatment for steroid-refractory celiac disease: a case report. Gastroenterology 2002;122:800-5.
  29. Costantino G, Torre A, Lo Presti M.A, Caruso R, Mazzon E, Fries W. Treatment of life-threatening type I refractory coeliac disease with long-term infliximab. Digestive and Liver Disease 2008;40:74-7.
  30. Tack GJ, Verbeek HM, Al-Toma Aet al. Evaluation of Cladribine treatment in refractory celiac disease type 2.World J Gastroenterol 2011;17:506-13.
  31. Al-Toma A, Verbeek, W. H, Hadithi, M, von Blomberg B. M, Mulder C. J. Cladribine therapy in refractory celiac disease with aberrant T cells. Clin Gastroenterol Hepatol 2006;4:1322-7.
  32. Mention JJ, Ben Ahmed M, Begue B, et al. Interleukin 15: a key to disrupted intraepithelial lymphocyte homeostasis and lymphomagenesis in celiac disease. Gastroenterology 2003;125:730-45.