Refractory coeliac disease: New updates on disease management
Diagnosis and therapy of refractory coeliac disease is hotly debated. The AGA has commissioned an update of recommendations for action.
Key takeaways from the updated practice guidelines:
- They were developed by a team of experts from the American Gastroenterological Association.
- The best practice advice (BPAs) are not guidelines, but are based on expert opinion and literature research.
- The BPAs presented here refer to the diagnosis and therapy of refractory coeliac disease.
BPA 1: Diagnostic procedure
The diagnosis of coeliac disease is not always straightforward and often involves a prolonged period of patient suffering. To confirm the diagnosis in people with persistent or recurrent symptoms suspicious of coeliac disease, the experts therefore recommend an assessment of all previous investigations, including serology, endoscopies and histologies.
BPA 2: Procedure in case of therapy resistance
If people with confirmed coeliac disease continue to have symptoms - either recurrent or persistent - the following steps should be taken:
- Further intake of gluten should be ruled out as it may be responsible for the symptoms.
- This should be accompanied by serological testing, stool examinations and dietary counselling.
- The experts recommend taking small intestinal biopsies to check for villous atrophy.
- If the symptoms persist, other causes must be ruled out. For example, autoimmune diseases, tropical sprue or drug-induced enteropathy are possible.
BPA 3: Do not neglect other causes of symptoms
If symptoms such as diarrhoea, flatulence or abdominal pain continue to occur after gluten intake has been ruled out, other causes for the symptoms should be considered. Other medical conditions such as inflammatory bowel disease, IBS or lactose or fructose intolerance may be responsible for the discomfort.
BPA 4 and 5: Determination of coeliac disease subtypes and differential diagnosis
Flow cytometry, immunohistochemistry and T-cell receptor rearrangement studies are suitable for determining the subtype of refractory coeliac disease. In this way, the differential diagnosis of T-cell lymphoma can also be excluded. Caution: Interpretation of the results should be done by a haematopathologist.
If refractory coeliac disease is suspected, the differential diagnoses of enteropathy-associated T-cell lymphoma or ulcerative jejunoileitis should be excluded, especially in type 2. This is ideally done by capsule endoscopy or CT/MRI.
BPA 6 and 7: Paying attention to nutrient intake
The diet of patients with refractory coeliac disease should always be checked for the intake of micro- and macronutrients. The albumin level can be helpful here. If there are nutrient deficiencies, these can and should be compensated for with food supplements or - if necessary - perenteral to correct the deficiency.
BPA 8: Drug therapy
The gold standard in first-line therapy for refractory coeliac disease, both type 1 and type 2, is corticosteroids. According to the experts, the most suitable are budesonide or prednisone.
BPA 9 and 10: Follow-up
The experts recommend regular monitoring of patients by a multidisciplinary team, which should always include gastroenterologists and dieticians. If the patients do not respond to the therapy, referral to a specialised centre or participation in clinical trials should be considered.
Conclusion for medical practice
Although the updated recommendations of the AGA do not represent a guideline, they are practical guidelines that can simplify the diagnosis and therapy as well as the management of refractory coeliac disease.
- Green PHR, Paski S, Ko CW, Rubio-Tapia A. AGA Clinical Practice Update on Management of Refractory Celiac Disease: Expert Review. Gastroenterology. 2022 Sep 19:S0016-5085(22)00967-2. doi: 10.1053/j.gastro.2022.07.086. Epub ahead of print. PMID: 36137844.