Special Article
Monitoring Non-responsive Patients with Celiac Disease

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Celiac disease

Celiac disease (Cd) is an inflammatory small intestinal disorder that is triggered by the ingestion of gluten proteins from wheat, barley and rye [1], [2], [3], [4], [5], [6], [7]. Its prevalence in the USA, most Western and middle Eastern countries ranges from 1:80–1:200 [3], [4], [5], [7], [8], [9], [10], [11]. Patients with Cd show a characteristic small intestinal (duodenal) histology of intraepithelial and subepithelial lymphocytic infiltration, villous atrophy and crypt hyperplasia. While

Diagnosis of celiac disease

The sensitivity of conventional video endoscopy to detect celiac disease is low. In a prospective study endoscopic markers for celiac disease, ie, loss and scalloping of duodenal folds, fissuring and mosaic pattern of the duodenal mucosa, had a sensitivity of only 50% and a specificity of 99.6%, with positive and negative predictive values of 60% and 99.4%, respectively [26]. Therefore, at least 3 biopsy specimens taken by “jumbo” forceps should be obtained from the distal duodenum of patients

Refractory celiac disease and intestinal lymphoma

Refractory celiac disease (RCd) can develop in 5–10% of adults with long-standing (often undetected) Cd. Patients do not respond to or relapse while on a strictly gluten-free diet. 158 Cd patients underwent follow-up small intestine biopsies within 2 years after starting a gluten-free diet [34]. Of 11 patients (7.0%) with persisting symptoms and villous atrophy who were considered to have RCd, five developed intestinal lymphoma.

The diagnosis of RCd can only be made when (inadvertent) gluten

Role of video endoscopy in celiac disease patients with persisting symptoms despite being on a gluten-free diet

There is a continuing debate on the benefits of video capsule endoscopy (VCE) over endoscopy and biopsy in patients with suspected or known (uncomplicated) Cd. Petroniene and colleagues [50] compared VCE to endoscopy in 10 patients with histologically proven villous atrophy and 10 non-celiac controls. Four blinded investigators assessed the VCE images, with an overall sensitivity of 70% and a specificity of 100% and a perfect interobserver agreement (kappa = 1.0). Two studies in patients with

Summary

Current data clearly suggest that VCE has an important role in the diagnostic workup of complicated Cd, especially in those cases with suspected RCd or EATL. This is supported by the high yield of relavant pathological findings in distal parts of the small intestine, such as severe ulceration and mucosal infiltration, by VCE compared to conventional upper GI endoscopy. In this setting, VCE allows for an assessment of the extent of small bowel involvement, detection of overt though often small

Acknowledgment

Part of the cited research of DS has been supported by research grants from the German Research Council (DFG) and the NIH, grant 1 R21 DK073254-01.

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      Non-responsive coeliac disease is however defined by a lack of initial response to a prescribed gluten free diet, or the recurrence of symptoms despite maintenance of a gluten free diet in a patient who initially responded [52]. The most common cause of non-responsive coeliac disease is either inadvertent or deliberate gluten exposure, which has been reported to occur in up to 50% of treated adult coeliac disease patients [53,54]. Although the presence of persisting circulating EMA or tTG antibodies strongly correlates with dietary mistakes [55], over 80% of patients with persisting Marsh 3 lesion whilst on gluten free diet do not have elevated levels of tTG IgA [52,56].

    • Enteroscopy in the Diagnosis and Management of Celiac Disease

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      A minimal, but continuous, intake of gluten can prevent mucosal recovery.68 However, when the dietary compliance is established by a dietician, and the patient still does not respond, the initial diagnosis of celiac disease must be reassessed, and other reasons for persisting symptoms (ie, pancreatic insufficiency, irritable bowel syndrome, bacterial overgrowth, inflammatory bowel disease, microscopic colitis, tropical sprue) must be ruled out.69 When clinical symptoms and histologic abnormalities persist or recur despite a strict adherence to the diet for more than 12 months, patients are defined as suffering from RCD.16,70–72

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