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Refractory celiac disease

Abstract

Celiac disease (CD) is a small intestinal inflammatory disorder characterized by an immune-mediated enteropathy triggered by the ingestion of wheat gluten or related rye and barley proteins in genetically predisposed individuals carrying the human leukocyte antigens (HLA)-DQ2 or-DQ8. Nonresponsive CD (NRCD) is a clinical diagnosis defined by the persistence of signs, symptoms, and/or laboratory abnormalities typical of CD despite adherence to a gluten-free diet for at least 6 months. One cause for NRCD is refractory CD (RCD), defined as the persistence of severe villous atrophy on small intestinal biopsy despite strict gluten withdrawal for at least 6 months with no evidence of other pathology. Although rare, RCD should be suspected in individuals with an established diagnosis of CD who fail to respond primarily or secondarily to a strict gluten-free diet, particularly if they manifest significant weight loss. A thorough evaluation must be performed to distinguish RCD from other causes of NRCD. RCD may be categorized into type I or type II. Type I RCD has a more favorable prognosis compared with type II and can often be managed with nutritional supplementation and possibly low level immunosuppressive therapy. Type II RCD carries a poor prognosis and is more likely to progress to life-threatening malnutrition or intestinal T-cell lymphoma. Immunosuppressive agents and, more recently, autologous stem cell transplant have been used to treat type II RCD.

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Correspondence to Ciarán P. Kelly.

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Abdallah, H., Leffler, D., Dennis, M. et al. Refractory celiac disease. Curr Gastroenterol Rep 9, 401–405 (2007). https://doi.org/10.1007/s11894-007-0049-5

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Keywords

  • Celiac Disease
  • Coeliac Disease
  • Small Intestinal Bacterial Overgrowth
  • Microscopic Colitis
  • Tropical Sprue