Current Gastroenterology Reports

, Volume 9, Issue 5, pp 401–405

Refractory celiac disease

  • Hani Abdallah
  • Daniel Leffler
  • Melinda Dennis
  • Ciarán P. Kelly
Article

DOI: 10.1007/s11894-007-0049-5

Cite this article as:
Abdallah, H., Leffler, D., Dennis, M. et al. Curr Gastroenterol Rep (2007) 9: 401. doi:10.1007/s11894-007-0049-5

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.

Copyright information

© Springer Science+Business Media, LLC 2007

Authors and Affiliations

  • Hani Abdallah
  • Daniel Leffler
  • Melinda Dennis
  • Ciarán P. Kelly
    • 1
  1. 1.The Celiac Center, Division of Gastroenterology, Beth Israel Deaconess Medical CenterHarvard Medical SchoolBostonUSA

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