Refractory celiac disease

Expert Rev Gastroenterol Hepatol. 2016;10(4):537-46. doi: 10.1586/17474124.2016.1124759. Epub 2015 Dec 16.


Refractory celiac disease (RCD) affects patients who have failed to heal after 6-12 months of a strict gluten-free diet (GFD) and when other causes of symptoms (including malignancy) have been ruled out. It may also occur in patients who previously had responded to a long-term GFD. RCD may be categorized as RCD1 (normal immunophenotype) and RCD2 (aberrant immunophenotype). RCD1 usually responds to a continued GFD, nutritional support, and therapeutic agents such as corticosteroids. In contrast, clinical response in RCD2 is incomplete and prognosis is often poor. RCD (particularly RCD2) is associated with serious complications, such as ulcerative jejunitis and enteropathy-associated T-cell lymphoma (EATL). Strict clinical and laboratory criteria should be used to diagnose RCD and specialized tests for aberrancy and clonality should be interpreted in the context of their sensitivity and specificity. Adequate nutritional support and anti-inflammatory treatment may even allow patients with RCD2 to attain a clinical remission.

Keywords: Celiac disease; gluten; immune disorder; refractory celiac disease; small intestine.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Anti-Inflammatory Agents / therapeutic use
  • Biopsy
  • Celiac Disease / diagnosis
  • Celiac Disease / diet therapy*
  • Celiac Disease / immunology
  • Combined Modality Therapy
  • Diagnosis, Differential
  • Diet, Gluten-Free*
  • Humans
  • Immunophenotyping / methods
  • Nutrition Assessment
  • Nutritional Support
  • Phenotype
  • Predictive Value of Tests
  • Remission Induction
  • Time Factors
  • Treatment Failure


  • Adrenal Cortex Hormones
  • Anti-Inflammatory Agents