Diagnosis and management of refractory celiac disease: a systematic review

Tunis Med. 2013 Aug-Sep;91(8-9):493-8.


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.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Celiac Disease / diagnosis*
  • Celiac Disease / epidemiology
  • Celiac Disease / therapy*
  • Diagnosis, Differential
  • Diet, Gluten-Free
  • Humans
  • Prognosis
  • Treatment Failure