Defining gluten refractory enteropathy

Objective The diagnosis of coeliac disease (CD) is based on the responsiven ess 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, often with different and confusing meanings. Methods On the basis of both a review of the literature and our clinical ex perience, we propose the following classification, A condition characterize d by a refractory enteropathy, not due to lymphoma, ulcerative jejunoileiti s or collagenous sprue, but in which gluten sensitivity has been shown prev iously or could be shown while the patients were on an immunosuppressive th erapy should be indicated as refractory CD. Those patients in whom gluten s ensitivity can be excluded should be considered to be affected by non-coeli ac refractory sprue. Finally, patients in whom the presence of CD cannot be either confirmed or excluded should be considered to be affected by undefi ned sprue. Results Twenty-four certain refractory patients are described in the litera ture. The data suggest a diagnosis of refractory CD in 13 patients, non-coe liac refractory sprue in three patients, and undefined sprue in eight patie nts. Conclusions We define refractory CD as a form of CD that no longer responds 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. Eur J Gastroenterol Hepatol 1 3:561-565 (C) 2001 Lippincott Williams & Wilkins.