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The diagnosis of celiac disease still requires a characteristic appearance on histological examination of mucosal biopsy specimens obtained from the small intestine. These findings include varying degrees of

Table 6.5

Strategy to Diagnose Patients on a Gluten-free Diet without a Confirmed Diagnosis of Celiac Disease

Who to challenge:

Patients started on gluten-free diet (GFD) without confirmatory histology Patients with an equivocal diagnosis or equivocal response to GFD Those who are HLA DQ2 and/or DQ8 positive and may have celiac disease

What to challenge with: Diet containing gradually increasing amounts of gluten Final amount will vary according to patient sensitivity Minimum of 10 g gluten/day (four slices of bread-one slice has ~2.25g) Maximum of as much gluten as tolerated Standard is four slices whole wheat bread a day

How long to challenge:

Varying opinions on this issue!

Depends on duration of GFD, amount of gluten in challenge Use serology and symptoms as a guide to timing the EGD with biopsy Even if patient remains asymptomatic and seronegative, EGD with biopsy recommended after two to six months

Other considerations: Avoid challenges in patients reporting severe reactions to gluten/wheat After long-term GFD expect a prolonged time to develop a response to gluten

Elderly and very young patients are not ideal candidates Many patients do not want to undergo challenge There are reports of relapses after a year on the diet villous atrophy, a change in the normal columnar appearance of the absorptive epithelium with crypt hyperplasia and increased numbers of IEL and lamina propria mononuclear cells. More minor degrees of histopathology may be missed on routine examination since the earliest changes are an increase in IEL. These features are not specific for celiac disease in that some or all of the histological findings can be found in tropical sprue, small intestinal bacterial overgrowth, viral gastroenteritis, intestinal lymphoma and severe acid-induced injury associated with a gastrinoma. Duodenal biopsies obtained at endoscopy are usually sufficient to make the diagnosis, but occasionally additional samples from the more distal jejunum are needed. It is recommended that multiple biopsies be obtained throughout the segments of the duodenum as the disease can be patchy. Newer endoscopic technologies including higher resolution imaging can be helpful in differentiating abnormal mucosa with fissuring, notching or scalloping and loss of villi from areas of more normal appearing mucosa [49].

With the availability of improved serologic testing, a second set of biopsies to confirm histological improvement or complete healing on a gluten-free diet is not necessary in most instances. A second endoscopy with biopsies should be reserved for patients failing to improve clinically or serologically in spite of adequate dietary treatment. The clinical improvement is rapid, while months to years are usually needed before complete histological resolution is seen [50].

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