Defining "gluten refractory enteropathy"

OBJECTIVE: The diagnosis of coeliac disease (CD) is based on the responsiveness of the enteropathy to a gluten-free diet (GFD). This implies that terms such as "non-responsive CD" and "refractory CD" are almost paradoxical: In spite of this, these terms are commonly used in the literature, ofter with different and confusing meanings. METHODS: On the basis of both a review of the literature and our clinical experience, we propose the following classification. A condition characterized by a refractory enteropathy, not due to lymphoma, ulcerative jejunoileitis or collagenous sprue, but in which gluten sensitivity has been shown previously or could be shown while the patients were on an immunosuppressive therapy should be indicated as refractory CD. Those patients in whom gluten sensitivity can be excluded should be considered to be affected by non-coeliac frefractory sprue. Finally, patients in whom the presence of CD cannot be either confirmed or excluded should be considered to be affected by undefined sprue. RESULTS: Twenty-four certain refractory patients are described in the literature. The data suggest a diagnosis of refractory CD in 13 pataients, non-coeliac refractory sprue in three patients, and undefined sprue in eight patients. CONCLUSIONS: We define refractory CD as a form of CD that no longer tresponds to a GFD. Non-coeliac refractory sprue is a condition unrelated to CD. It could be either an independent condition or a common end point of different enteropathies.