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Refractory celiac disease.

Abdul R Rishi1, Alberto Rubio-Tapia1, Joseph A Murray1

  • 1a Division of Gastroenterology and Hepatology , Mayo Clinic , Rochester , MN , USA.

Expert Review of Gastroenterology & Hepatology
|November 26, 2015
PubMed
Summary
This summary is machine-generated.

Refractory celiac disease (RCD) is diagnosed when a gluten-free diet fails. RCD2, a subtype, has a poor prognosis but may improve with nutritional support and anti-inflammatory treatments.

Keywords:
Celiac diseaseglutenimmune disorderrefractory celiac diseasesmall intestine

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Area of Science:

  • Gastroenterology
  • Immunology
  • Oncology

Background:

  • Refractory celiac disease (RCD) impacts patients unresponsive to a strict gluten-free diet (GFD) for 6-12 months.
  • RCD can occur despite previous GFD response, necessitating exclusion of other conditions like malignancy.
  • RCD is classified into RCD1 (normal immunophenotype) and RCD2 (aberrant immunophenotype).

Purpose of the Study:

  • To define refractory celiac disease (RCD) and its subtypes.
  • To outline diagnostic criteria and associated complications.
  • To discuss management strategies and prognosis for RCD subtypes.

Main Methods:

  • Review of clinical and laboratory criteria for RCD diagnosis.
  • Evaluation of immunophenotype and clonality testing in RCD.
  • Analysis of treatment responses and complications in RCD subtypes.

Main Results:

  • RCD1 typically responds to continued GFD, nutritional support, and corticosteroids.
  • RCD2 exhibits incomplete clinical response and a generally poor prognosis.
  • RCD, especially RCD2, is linked to severe complications like ulcerative jejunitis and enteropathy-associated T-cell lymphoma (EATL).

Conclusions:

  • Accurate RCD diagnosis requires strict clinical and laboratory criteria, with careful interpretation of specialized tests.
  • While RCD2 poses significant challenges, adequate nutritional support and anti-inflammatory therapies may lead to clinical remission.