The American Gastroenterological Association (AGA) Institute Clinical Practice Updates Committee (CPUC) and the AGA governing board released 10 best practice advice statements for diagnosing and managing refractory celiac disease.
Best Practice Advice Statement #1: Obtain Diagnostic Confirmation of Celiac Disease
Serologic testing leads to endoscopy and duodenal biopsy to confirm initial diagnosis of celiac disease. Pathologic histologic findings must include villous atrophy, intraepithelial lymphocytosis, and crypt hyperplasia. However, these findings are not exclusive to celiac disease. If diagnostic testing remains inconclusive, further testing for HLA haplotypes DQ2 or DQ8 may provide confirmation of celiac disease.
Best Practice Advice Statement #2: Exclude Ongoing Gluten Ingestion as Possible Cause of Recurring Symptoms
Patients with nonresponsive celiac disease must completely exclude gluten ingestion (whether intentional or inadvertent) from their diet. Further serologic testing, dietitian consultation, and biomarker testing for immunogenic peptides in stool and urine help to confirm persistent gluten ingestion.
Esophagogastroduodenoscopy and small bowel biopsies confirm persistent villous atrophy. Differential diagnosis for other conditions that can cause villous atrophy should include medication-induced enteropathy, tropical sprue, immunodeficiency conditions, and autoimmune enteropathy.
Best Practice Advice Statement #3: Perform Systemic Evaluation to Rule Out Other Potential Causes of Refractory Symptoms
Systemic evaluation to rule out other conditions that might contribute to persistent symptoms include the following:
- breath tests to rule out lactose and fructose intolerance as well as small intestinal bacterial overgrowth (SIBO)
- colonoscopy to rule out microscopic colitis and inflammatory bowel disease in patients with confirmed celiac disease who experience persistent or recurrent diarrhea
- initiation of a low-fermentable oligo-, di-, and monosaccharides and polyols (FODMAP) diet if functional bowel disorders such as irritable bowel syndrome are suspected
- treatment of pancreatic insufficiency with gluten-free pancreatic enzyme supplements
Clinicians may strongly suspect refractory celiac disease in cases of malabsorption in the absence of malignancies or other possible explanations.
Best Practice Advice Statement #4: Determine Refractory Celiac Disease Subtype
Flow cytometry, immunohistochemistry, and T-cell receptor rearrangement studies differentiate between type 1 and type 2 refractory celiac disease while ruling out enteropathy-associated T-cell lymphoma (EATL). An experienced hematopathologist confirms the presence of normal intraepithelial lymphocytes in type 1 refractory celiac disease or aberrant, clonal intraepithelial lymphocytes in type 2.
Best Practice Advice Statement #5: Rule Out EATL and Ulcerative Jejunoileitis
If type 2 refractory celiac disease is confirmed, it is recommended to perform small bowel imaging with capsule endoscopy complemented by computed tomography or magnetic resonance enterography to rule out comorbid EATL and ulcerative jejunoileitis.
Best Practice Advice Statement #6: Perform Comprehensive Nutritional Assessment
Patients diagnosed with refractory celiac disease must undergo a comprehensive nutritional assessment to assess for micronutrient and macronutrient deficiencies as well as albumin levels.
Best Practice Advice Statement #7: Correct Nutritional Deficiencies
Based on the comprehensive nutritional assessment results, suggest oral supplementation and/or enteral or parenteral nutritional support to correct existing nutritional deficiencies according to severity of malabsorption.
Best Practice Advice Statement #8: Use Corticosteroids as First-Line Treatment
First-line therapy of choice for either type 1 or type 2 refractory celiac disease involves use of glucocorticosteroids, most frequently open-capsule budesonide or prednisone. Approximately 80% to 90% of patients with refractory celiac disease exhibit adequate clinical response to open-capsule budesonide or prednisone with higher clinical responses observed in patients with type 1 refractory celiac disease.
The optimal choice for second-line therapy is not known yet; however, adding immunosuppressants such as azathioprine, tioguanine, and mercaptopurine to steroid treatment seems to increase efficacy in patients with type 1 refractory celiac disease.
Best Practice Advice Statement #9: Perform Routine Follow-Ups of Patients Diagnosed with Refractory Celiac Disease
A multidisciplinary team of experts, including a gastroenterologist and dietitian who are familiar with management of refractory celiac disease, must perform ongoing, regular follow-ups to assess patient clinical and histologic responses to treatment.
Hypoalbuminemia strongly predicts mortality and should be monitored frequently during all follow-up visits.
Best Practice Advice Statement #10: Refer Patients to Centers Conducting Clinical Trials or with Other Means of Management
If clinical and histologic findings following repeat intestinal biopsy after 6 months of steroid therapy indicate that a patient is unresponsive to treatment, clinicians should try another alternative medication or intervention. If repeat biopsy indicates continued nonresponse to alternative treatment, clinicians should refer these patients to a center with expertise for management of refractory celiac disease or to clinics performing clinical trials.
Depending on each individual case, small bowel imaging (capsule endoscopy, MRI or CT enterography, and 18F-fluorodeoxyglucose positron-emission tomography (PET) scans may be warranted to monitor for suspected lymphoma.
“Diagnosis and management of RCD remains challenging due to the rarity of the condition and the absence of a reference standard diagnostic marker,” the guideline authors wrote. “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.”
Disclosures: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.
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