REVIEW ARTICLE - MANAGEMENT OF PATIENTS WITH NONRESPONSIVE CELIAC-DISEASE

A substantial minority of patients with coeliac disease (estimated at anything between 7 and 30%) fail to respond to treatment with a gluten -free diet, Nonresponsiveness may be primary, that is when the patient fails to respond to treatment following initial diagnosis, or seconda ry, when a patient who has previously had a documented response to glu ten exclusion becomes non-responsive to therapy, The commonest cause o f non-responsiveness is continued gluten ingestion, either voluntary o r inadvertent, Other causes to be considered include intolerances to d ietary constituents other than gluten (e.g. milk, soya), pancreatic in sufficiency, enteropathy-associated T-cell lymphoma and ulcerative jej unitis. There is some evidence that ulcerative jejunitis is, in fact, a manifestation of lymphoma, The most important steps in the managemen t of the non-responsive coeliac patient are (a) to determine whether t he patient is indeed coeliac, (b) to exclude lymphoma and (c) to estab lish the cause of the nonresponsiveness, In those coeliac patients wit h no demonstrable cause for non-responsiveness, a variety of therapeut ic stratagems (mostly based on small, uncontrolled studies) have been described; these include elimination diets, dietary supplementation wi th zinc and copper, and pharmacological therapy in the form of steroid s, azathioprine and cyclosporin, In a minority of non-responsive patie nts, the clinical course is characterized by a rapid decline, and tota l parenteral nutrition is required, None of the therapies described ab ove has been subjected, to rigorous controlled studies. The precise me chanisms of non-responsiveness in coeliac patients need to be unravell ed before rational therapeutic approaches can be established.