Role of immunosuppressive therapy in refractory sprue-like disease

Patients with malabsorption and histological findings consistent with celia c disease, who are unresponsive to gluten free diet, and in whom other caus es of flat mucosa have been excluded, are considered to suffer from so call ed unclassified or refractory sprue, Although the true nature of this condi tion needs to be further elucidated, it is known to represent a difficult t herapeutical problem with potentially fatal course. Herein, me report a pat ient with refractory sprue-like disease who after failing to respond to cor ticosteroids and TPN was in a critical condition. He responded promptly to cyclosporine and made a remarkable recovery. In contrast to previous report s, the cyclosporine treatment in this patient was pursued only for 1 month, whereupon the patient turned responsive to steroids. Subsequent treatment with azathioprine allowed corticosteroids to be reduced to a low maintenanc e dose and eventually all drugs could be discontinued without reappearance of symptoms. Cyclosporine therapy might be lifesaving in occasional patient s with refractory sprue-like disease and it may result in reversal of stero id resistance. moreover azathioprine seems to have a steroid sparing effect in this setting. Short term immunosuppressive treatment may have an advant age of lower risk for drug related side effects. (C) 1999 by Am. Coll. of G astroenterology.