Refractory celiac disease

ABSTRACT

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
• refractory celiac disease
• gluten
• immune disorder
• small intestine

Financial & competing interests’ disclosure

JA Murray has received grant support from Alba Therapeutics, Alvine Pharmaceuticals, Inc., and the National Institutes of Health. He serves on the advisory board of Alvine Pharmaceuticals, Inc. and Celimmune, LLC and is a consultant to AMAG Pharmaceuticals, Entera Health, Inc, Sonomaceuticals, LLC, BioLineRx, GlaxoSmithKline (GSK), Genentech, Glenmark Pharmaceuticals Ltd, and Boehringer Ingelheim. He has a patent with Miomics and receives royalties from Torax. He received payment for the development of educational presentations from National Foundation Celiac Awareness. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.