Management of Refractory Celiac Disease

Karoly Horvath, MD, PhD, Alessio Fasano, MD, Division of Pediatric Gastroenterology and Nutrition, Department of Pediatrics, University of Maryland School of Medicine, Baltimore, Maryland.

In This Article


The diagnosis of RS can be made only after other gastrointestinal diseases have been ruled out ( Table 2 ). However, the priority is to assure that the patient adheres to a strict gluten-free diet. The normalization of the serology test (antigliadin, antiendomysium, and antitissue transglutaminase antibodies) can be a sign of adherence to the appropriate diet. However, the tests currently available are not sensitive enough to detect small gluten exposure; therefore, a nutritional consultation warrants consideration as the second step in management.

If compliance to diet is adequate, additional laboratory and histologic studies are warranted. After the initial screening, other causes of chronic diarrhea and complications of CD must be considered in the differential diagnosis. The most evident consideration should be whether there is lactose malabsorption. Low or absent lactase activity is a well-known problem in newly diagnosed celiac cases. Patients with long-term CD can have pancreatic insufficiency. Collagenous colitis and lymphocytic colitis can accompany CD and can cause chronic diarrhea that is nonresponsive to a gluten-free diet.

Some patients with RS symptoms may have intolerance to other, nongluten food antigens. One of the RS cases reported on by Robert and colleagues involved beef-protein intolerance that improved on beef-free diet.

Serologic tests are also performed to examine for the presence of antienterocyte antibodies. A small proportion of patients appear to have an adult form of autoimmune enteropathy, characterized by the presence of antienterocyte antibodies. Autoimmune enteropathy is well described in children without CD. Corazza and colleagues reported 2 adult cases; however, neither had serologic evidence of CD. There are no other published studies examining for the presence of antienterocyte antibodies in patients diagnosed with RS.


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