AGA releases 10 best practice statements for management of refractory celiac disease
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In an expert review published in Gastroenterology, AGA provided updated guidance for the diagnosis and management of refractory celiac disease, including supportive and therapeutic treatment options and recommendations for monitoring.
“This new clinical practice update on management of refractory celiac disease presents a step-by-step clinical approach for patients with celiac disease following a gluten-free diet with persistent or recurrent symptoms and the most up-to-date information about tests and treatments for patients with refractory celiac disease,” Alberto Rubio-Tapia, MD, senior author of the update and director of the Celiac Disease Program at the Cleveland Clinic, told Healio. “This AGA clinical practice update would be very helpful to clinicians to improve care of patients with refractory celiac disease.”
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The best practice statements, which were based on review of published literature and expert opinion, were approved by the AGA Institute Clinical Practice Updates Committee and the AGA Governing Board. Highlights include:
- Initial diagnosis of celiac disease should be confirmed by review of diagnostic testing, including serologies, endoscopies and histologic findings, in patients believed to have celiac disease.
- In patients with nonresponsive celiac disease, ongoing gluten ingestion should be excluded based on serologic testing, dietician review and detection of immunogenic peptides in stool or urine. Villous atrophy may be identified with esophagogastroduodenoscopy and small bowel biopsies.
- In patients with nonresponsive celiac disease, systematic evaluation for other causes of symptoms, which may include functional bowel disorders, microscopic colitis, pancreatic insufficiency, inflammatory bowel disease, lactose or fructose intolerance, and small intestinal bacterial overgrowth, should be performed after exclusion of gluten ingestion.
- To distinguish between subtypes of refractory celiac disease and exclude enteropathy-associated T-cell lymphoma, the use of flow cytometry, immunohistochemistry and T-cell receptor rearrangement studies is recommended.
- Small bowel imaging with capsule endoscopy and CT or magnetic resonance enterography should be performed to exclude enteropathy-associated T-cell lymphoma and ulcerative jejunoileitis at initial diagnosis of type 2 refractory celiac disease.
- In patients with refractory celiac disease, a detailed nutritional assessment with investigation of micronutrient and macronutrient deficiencies should be performed. An assessment of albumin may be an independent prognostic factor.
- Oral supplements and/or enteral support should be used to correct deficiencies in macronutrients and micronutrients. In patients with severe malnutrition because of malabsorption, parenteral nutrition should be considered.
- Corticosteroids, including open-capsule budesonide or prednisone, should be used as first-line therapy in type 1 or type 2 refractory celiac disease.
- Patient monitoring by a multidisciplinary team, including gastroenterologists and dietitians, is necessary to evaluate clinical and histologic response to therapy.
- Referral to a center with expertise for management or evaluation for inclusion in clinical trials may be beneficial for patients with refractory celiac disease who do not respond to steroids.
“Diagnosis and management of [refractory celiac disease (RCD)] remains challenging due to the rarity of the condition and the absence of a reference standard diagnostic marker,” the authors concluded. “Ongoing prospective and comparative studies are needed to define proper diagnostic criteria for well-classified RCD patients and to identify optimal management strategies for this rare condition.”
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