Celiac Disease (Sprue) Guidelines

Updated: Jul 25, 2019
  • Author: Stephan U Goebel, MD; Chief Editor: BS Anand, MD  more...
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Guidelines

Guidelines Summary

In June 2019, the European Society for the Study of Coeliac Disease (ESsCD) released updated guidelines for the management of celiac disease (CD) and other gluten-related disorders in adults and children. [62, 63] Their strong CD recommendations are outlined below.

Who should be tested for celiac disease?

Adult patients with symptoms, signs, or laboratory evidence suggestive of malabsorption: Test with serology for CD.

Patients with unexplained elevation of serum aminotransferase levels: Exclude CD.

Patients with type 1 diabetes: Screen regularly for CD.

Workup

Serology

IgA-TG2 (immunoglobulin A-tissue transglutaminase 2) antibody is the preferred single test for CD detection at any age. Concurrently measure total IgA level with serology testing to determine whether IgA levels are sufficient.

At diagnosis and follow-up, perform IgG-based testing (IgG-DGPs [deamidated gliadin peptides] or IgG-TG2) in patients with selective total IgA-deficiency.

Perform all diagnostic serologic testing while patients are on a gluten-containing diet.

Antibodies directed against native gliadin (AGA) are not recommended for primary CD detection.

Endoscopy and histopathology

When CD is suspected, obtain biopsies even with a normal endoscopic appearance of the duodenum.

Duodenal biopsy is an essential component of the diagnostic evaluation for adults with suspected CD and is recommended to confirm the diagnosis. Multiple duodenal biopsies (at least four of the second part of duodenum) are recommended for confirmation.

An increase in intraepithelial lymphocyte (IEL) infiltration in the absence of villus atrophy (VA) in duodenal biopsies (Marsh 1) is not specific for CD; exclude other causes.

Helicobacter pylori infection is often associated with Marsh 1 histology; its eradication may normalize the duodenal IEL count. Obtain concomitant gastric biopsies or perform serology when H pylori is suspected.

If CD is highly suspected, perform duodenal biopsy even if the serology is negative.

HLA-DQ2/8 typing

Avoid routine use of HLA-DQ2/DQ8 testing in the initial diagnosis of CD. Include the results of such testing with a caution that patients at risk should be serologically tested for CD without changing their diet.

Use HLA-DQ2/DQ8 testing to rule out CD in selected clinical situations, including: (a) Marsh 1-2 histology in seronegative patients; (b) evaluation of patients not tested for CD before initiation on a gluten-free diet (GFD); (c) discrepant results of celiac-specific serology and histology.

Other diagnostic tests

Video capsule endoscopy (VCE) is not used for the initial diagnosis of CD except for patients with positive celiac-specific serology who are unwilling/unable to undergo endoscopy with biopsy. VCE is important in detecting complications associated with CD.

Intestinal-permeability tests are neither sensitive nor specific and are not recommended for CD diagnosis.

Serum intestinal fatty acid binding protein (I-FABP) might be useful in identifying dietary nonadherence and unintentional gluten intake.

Diagnostic confirmation

Diagnostic confirmation of CD in adults and in some children should be based on clinical data, positive serology, and duodenal histology.

Improvement of symptoms or exacerbation after gluten re-introduction has a very low predictive value for CD and should not be used for diagnosis in the absence of other supportive evidence.

A positive CD-specific serology in patients with VA confirms the CD diagnosis.

In case of an elevated TG2-titer and normal histology, a pathologist familiar with CD should review the biopsies. Repeat the biopsy after gluten challenge if the patient was not on gluten-containing diet before testing. HLA-DQ2/8 typing is mandatory. Testing for other antibodies (eg, DGP and/or endomysium [EMA]) may be of added value.

Seronegative CD requires careful assessment with HLA-DQ2/8 testing and a response to a GFD after excluding other causes of seronegative VA. Coeliac serology, both IgA- and IgG-based, should be negative.

In patients who are already following GFD prior to testing, serology and HLA typing are needed. If serology is positive, then biopsy is the next step. Perform a gluten challenge when serology is negative but HLA DQ2/DQ8 is positive.

Dietary management

Patients with CD should adhere to a lifelong GFD. The majority of CD patients safely tolerate oats; introduction of oats into the diet should be cautious, and monitor patients for possible adverse reaction.

Refer patients with CD to a dietitian who is well-trained concerning CD to get a detailed nutritional assessment, education on the GFD, and subsequent monitoring.

Newly diagnosed adult CD patients should undergo testing to uncover deficiencies of essential micronutrients (eg, iron, folic acid, vitamins D and B12).

Advise patients to eat a high-fiber diet supplemented with whole-grain rice, maize, potatoes, and ample vegetables.

Follow-up

Monitor CD patients regularly for persistent or new symptoms, adherence to GFD, and assessment for complications. Base monitoring of GFD adherence on a combination of history and serology. Monitoring of CD patients should include verification of the normalization of laboratory abnormalities detected during the initial investigation.

Dietary revision should be performed by a dietitian with special expertise in CD especially in slow-responders to exclude gluten contamination.

A normal anti-TG2 level at follow-up does not predict recovery of VA.

A follow-up duodenal biopsy is recommended for monitoring in cases of lack of clinical response or relapse of symptoms despite a GFD.

CD patients who are known to be hyposplenic should receive the pneumococcal vaccine.

Measure bone density in those at high risk of osteoporosis at diagnosis, especially in those with malabsorption or those at high risk if there is a long delay in diagnosis, or there are clinical presentations suggestive of bone disease. In others, not later than age 30-35 years and then to be repeated at 5-year intervals. Use a shorter interval (2-3 years) in case of low bone density on index measurement, evidence of ongoing VA, or poor dietary adherence.

Slow-responders and refractory CD (RCD)

Carefully evaluate patients showing slow response to exclude dietary inconsistencies and also identify other specific etiologies. The evaluation should include review of the initial diagnosis, celiac serology, a dietary review, and a follow-up duodenal biopsy.

Distinguish RCD-I from RCD-II to select appropriate management and determine the prognosis. T-cell flow cytometry is the most reliable method to make a distinction between RCD-I and RCD-II.

Closely monitor the nutritional status of RCD patients. Nutritional support including parenteral nutrition forms an essential part of the management.

Confidently exclude enteropathy-associated T cell lymphoma (EATL) before initiating pharmacotherapy in RCD-II. RCD-II patients need to be treated in referral centers by an experienced gastroenterologist in CD with a hematologist.

Exclude EATL in any CD patient with abdominal pain, fever, obstruction, anemia, gastrointestinal bleeding, or unexplained weight loss.

In EATL, debulking surgery for large ulcerated small bowel tumors is recommended for limiting the risk of perforation or bleeding during chemotherapy.

Special issues about children and adolescents

Duodenal histology in some children is recommended to confirm the diagnosis of CD.

Introduce a GFD only when the CD diagnosis has been made conclusively.

A gradual, structured transfer of medical care of an adolescent with CD to adult care is recommended. Include as the minimum written information on the base of diagnosis, follow-up, anthropometric data, comorbidities, and dietary adherence.