Adult autoimmune enteropathy treated successfully with tacrolimus

BACKGROUND: Autoimmune enteropathy is a life-threatening, chronic disease of the small bowel mucosa, which generally responds well to steroids. Treatment requires long-term immunosuppression, and steroid-sparing treatment strategies are desirable. Azathioprine and cyclosporine A have limitations, however alternatives have not been described in adults. CASE REPORT: We present the case of a 54-year-old male patient with autoimmune enteropathy who responded initially to a standard treatment with steroids, but was dependent on 30 mg prednisolone. Medical treatment was changed to tacrolimus after renal function deteriorated under treatment with cyclosporine A. Under this regimen, small bowel histology normalized and the clinical condition is stable after 2 years of introduction of tacrolimus. CONCLUSION: This constitutes the first report of effective treatment of adult autoimmune enteropathy with tacrolimus, a substance with a similar mode of action to cyclosporine, but with fewer side effects and improved bioavailability.