Update on European Guidelines on Celiac Disease & Other Gluten-Related Disorders

August, 2019

Updated recommendations on the management of celiac disease (CD) and gluten-related disorders were recently published by the European Society for the Study of Coeliac Disease. A multi-national group of experts including both pediatric and adult gastroenterologists conducted a review of current guidelines, and a literature review of relevant articles published from 1990 to the present. The authors note that new guidelines were needed because previously available international guidelines were outdated and much new data has become available for consideration and incorporation.

Note: Decisions regarding care and follow-up should always be made on an individual basis in consultation with your personal healthcare team.

Selected highlights and key points (extracted or summarized from original article)

(The full article can be found here. https://journals.sagepub.com/doi/10.1177/2050640619844125)

• Recommendations on who should have screening blood work done for CD:
  • Adult patients with symptoms, signs or lab evidence that suggests malabsorption.
  • Patients with: IBS,  microscopic colitis, Hashimotos thyroiditis and Graves disease, osteopenia/osteoporosis, unexplained ataxia or peripheral neuropathy,  recurrent sores in the mouth or dental enamel defects, infertility, recurrent miscarriage,  late menarche, early menopause, chronic fatigue syndrome, epilepsy, headaches including migraines,  mood disorders, attention deficit disorders/cognitive impairment,  psoriasis or other skin lesions, Down or Turner’s syndrome.
  • Screening of asymptomatic first-degree family members of CD patients is recommended.
  • Individuals with type 1 Diabetes should be screened regularly for CD.

• Alternative screening tests:
• • Salivary and fecal screening tests do not yet have enough evidence to support their use in accurate screening.

• Endoscopy:
  • Multiple biopsies of the duodenum are recommended to confirm the diagnosis of CD. Addition of two biopsies of the duodenal bulb might increase the diagnostic yield.

• Education and follow-up:
• • Newly diagnosed individuals should be referred to a dietitian, preferably one with specialized knowledge.
  • Dietary adherence improves by having regular follow-up in the setting of a specialist celiac clinic.
  • Patients should be encouraged to join national celiac societies or other disease-specific support groups.
  • There is extensive evidence to support the central role of a dietitian in slow-responder patients or if gluten contamination is suspected.

• Nutritional deficiencies/excesses in celiac disease and the gluten-free diet (GFD):
• • Newly diagnosed adults with CD should undergo testing to uncover deficiencies of essential micronutrients.
  • Patients should be advised to eat a high fiber diet.
  • Iron deficiency is present in 7-80% of celiac patients at diagnosis.
  • CD is present in 2-5% of patients with iron deficiency anemia
  • Vitamin B12 deficiency is present in 5-41% of untreated CD cases; it is typically corrected with a GFD, but should be treated with a supplement in the short term.
  • Children on a GFD were found to have increased intake of simple sugars, fats and proteins, and higher energy intake.

• Celiac disease follow-up in adults:
• • In the first year after diagnosis, follow-up should be frequent to optimize chance of dietary adherence, provide psychological support, and to optimally motivate adaptation to living gluten-free.
  • CD patients should be monitored regularly for persistent or new symptoms, adherence to the GFD, and assessment for complications.
  • Once the disease is stable and the patients manage their diet without problems, annual or biennial follow-ups should be initiated.
  • Monitoring of adherence to the GFD should be based on a combination of history and serology.
  • Bone density testing should be done in individuals at high risk of osteoporosis. In others, it should be done at not later than age 30-35.
  • A normal anti-TG2 level at follow-up does not predict recovery of intestinal cell damage. A degree of villous atrophy is present in about 40% of patients who are re-biopsied at one year, despite good dietary compliance.
  • Currently, there are no studies indicating an absolute necessity for performing routine follow-up biopsy for all patients. However, symptomatic patients may need repeated biopsies to rule out refractory celiac disease (RCD) or other complications.

• “Slow Responders”:
  • A considerable percentage of adult CD patients (between 7 and 30%) continue to have persistent symptoms, signs or laboratory abnormalities despite at least 6–12 months on a gluten-free diet. These are regarded as slow-responders. The use of the term non-responsive CDto denote these patients is discouraged because most of them will improve over time on a strict GFD, or they have another treatable cause for their complaints.
  • In patients with confirmed CD the ingestion of gluten, either purposeful or inadvertent, is the most common cause of slow-response, being identified in 35–50% of cases. Therefore, a careful dietary evaluation is the next important step in the assessment.
  • Recommended evaluation should include a review of initial diagnosis, celiac blood work, dietary review, and follow-up biopsy.

• Refractory celiac disease:
  • Defined as persistent or recurrent symptoms and signs of malabsorption with villous atrophy despite following a strict gluten-free diet for over 12 months.
  • There is no curative treatment. Refractory CD must be diagnosed and treated in consultation with a physician/medical team.

• Celiac disease testing in kids and adolescents:
  • Testing should be offered to those who are asymptomatic but have an increased risk of CD such as due to Type 1 diabetes, Down syndrome, autoimmune thyroid disease, Turner syndrome, Williams syndrome, selective IgA deficiency, autoimmune liver disease and first degree relatives with CD.

• Non-coeliac gluten sensitivity (NCGS):
  • The diagnosis of NCGS should be excluded if symptoms do not improve after 6 weeks on a gluten-free diet.
  • It is acknowledged that a less-stringent gluten-free diet might be sufficient compared to those with CD; however this is a “conditional” recommendation which is currently supported by only a low level of evidence.

• Celiac disease-related skin disorders and disorders relating to the mouth and teeth:
  • A gluten-free diet should be considered in patients with psoriasis who have serological evidence of gluten intolerance even in the absence of clinical signs of celiac disease.
  • Dentists who encounter oral symptoms should inquire about the presence of autoimmune diseases which would increase the probability of having celiac disease. If CD is suspected, the patient’s primary care doctor or specialist should be consulted.

• Neuro-psychiatric manifestations related to gluten:
  • In established celiac disease, there is a 10-22% prevalence of neurological dysfunction reported.
  • “Neuroceliac” disease may easily go unrecognized.
  • Neither the causative factors nor the physiological mechanisms of neurological involvement in CD are understood at this time.
  • Gluten ataxia is the most frequently reported neurological disturbance in celiac disease. (Ataxia is a condition of the brain that affects balance and coordination.)
  • Up to 25% of people with celiac disease who are on a gluten-free diet have neurophysiological evidence of peripheral neuropathy.
  • New diagnostic tools are becoming available which will enable identification of patients with neuro-CD.

• Areas of uncertainty and future research:
  • Regarding screening. There is a lack of understanding of the natural history of undiagnosed celiac disease. This needs further clarification to justify screening of asymptomatic persons.
  • Recommended time intervals for doing repeat testing in high risk populations needs to be determined.
  • The debate on the diagnosis of CD without biopsy needs more clarification. Also, the indication for and timing of biopsy at follow-up should be further explored.
  • Future research on non-celiac gluten sensitivity should explore genetic background, histological characteristics, susceptibility, and risk factors.
  • Future research on non-celiac gluten sensitivity should explore developing reliable biomarkers.
  • Regarding neuro-celiac disease, more data are needed to clarify the gluten-brain link and to develop preventive and therapeutic strategies.
  • Development of non-dietary therapies might alleviate symptoms, especially after inadvertent gluten exposure.
  • An effective replacement for the gluten-free diet as treatment could greatly enhance quality of life of patients who struggle with the diet.